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Sample records for anca mihaela lazar

  1. Lazar Gulkowitschi mõistelooline meetod : (Begriffsgeschichte) / Urmas Nõmmik

    Index Scriptorium Estoniae

    Nõmmik, Urmas, 1975-

    2009-01-01

    Aastatel 1934-1941 Tartu Ülikooli filosoofiateaduskonnas juudi teaduste õppetooli juhatanud Lazar Gulkowitschi väljatöötatud eelkõige filoloogilisest mõiste- ehk ideedeloo meetodist ning seda meetodit sisaldavatest uurimistöödest

  2. Pathogenesis of PR3-ANCA associated vasculitis

    NARCIS (Netherlands)

    Kallenberg, C. G. M.

    2008-01-01

    Wegener's Granulomatosis (WG) is closely associated with antineutrophil cytoplasmic autoantibodies (ANCA), particularly those directed to proteinase 3 (PR3). ANCA directed to myeloperoxidase (MPO) are associated with microscopic polyangiitis (MPA) and the Churg Strauss syndrome. PR3-ANCA associated

  3. Detection of antineutrophil cytoplasmic antibodies (ANCAs)

    DEFF Research Database (Denmark)

    Damoiseaux, Jan; Csernok, Elena; Rasmussen, Niels

    2017-01-01

    of diagnosis) from 251 patients with ANCA-associated vasculitis (AAV), including granulomatosis with polyangiitis and microscopic polyangiitis, and from 924 disease controls were tested for the presence of cytoplasmic pattern/perinuclear pattern and atypical ANCA (A-ANCA) by indirect immunofluorescence (IIF...

  4. Epidemiology of ANCA associated vasculitis

    Directory of Open Access Journals (Sweden)

    Wenche Koldingsnes

    2009-10-01

    Full Text Available ANCA associated vasculitis (AAV comprises three syndromes with systemic vasculitis (Wegener’s granulomatosis (WG, Churg Strauss syndrome (CSS and icroscopic polyangiitis (MPA, which all involve small and medium sized vessels and are associated with antibodies against cytoplasmatic antibodies in neutrophils (ANCA. Polyarteritis nodosa (PAN is included in this review as it also affects medium sized vessels, and has many clinical findings in common with the AAV.Since the recognition of ANCA, increasing data have become available on the epidemiology of these vasculitidis. WG constitutes half of the AAV and its prevalence has increased from 30/million in the late 1980’s in the USA to 160/million in this century in northern Europe. The prevalence for the whole group of primary systemic vasculitides is now 300/million in Sweden. The annual incidence of WG increased from 6.0/million to 14/million during the 1990’s in Tromsø, but it is unknown if this is a true increase or the result of an increased awareness of the diagnosis. For the whole group of AAV, the annual incidence in most more recent studies is relatively constant over time and by geographical location, ranging from 13 to 21/million. Nonetheless there are interesting differences in the prevalence of specific vasculitis between different geographical areas, as well as for sub specificities of ANCA.There seems to be a South-North gradient for WG and PR3-ANCA with high figures reported from northern Europe and southern New Zealand. In European studies WG is 90% PR3-ANCA positive. MPA which is predominantly MPO-ANCA associated are more frequent in the Mediterranean countries and also has an increasing gradient towards east-Asia, as almost all AAV in China and Japan are diagnosed as MPA, predominantly MPO-ANCA positive.There are also some ethnic and gender differences. WG is most prevalent among Caucasians in the USA and in people with European ancestors in Paris and in New Zealand, less

  5. Seal-die of Prince Lazar from Rudnik

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    Radičević Dejan

    2016-01-01

    Full Text Available The medieval settlement on the Rudnik Mountain was established, most probably, in the final decades of the 13th century. Soon it evolved into one of the best known mining and commercial centres in the Serbian state and reached its peak during the 14th and in the first half of the 15th century. The importance of Rudnik in the medieval period is confirmed by numerous material traces in the field. The most important discoveries in the course of archaeological investigations carried out since 2009 have been encountered in the area called Drenje, not far from the centre of the town of Rudnik (figs. 1, 2.Three churches (two Orthodox and one Roman Catholic, as well as many profane structures dated to the time of a thriving medieval Rudnik have so far been discovered. It indicates that in that area are the remains of the main settlement and the medieval market place of Rudnik, known from written sources. Among other structures at the site of Drenje, in the garden of S. Markovi}, the remains of rather a large building consisting, according to present data, of at least two rooms, have been investigated during the past three years (fig. 3. Important for dating the structure is a coin of the Hungarian king Sigismund of Luxembourg (1387-1437, discovered on the floor. Traces of an earlier phase of life were encountered under the structure (fig. 5. Coins have been found in two earlier pits. In the soil inside pit 11 a coin of the Hungarian king Charles Robert (1308-1342 was found, while in the top level of pit 2 a coin attributed to Prince Lazar (around 1370-1389 was found. Also from pit 2 came a, so far, unique archaeological find in our territory. It has been explained as a seal-die used for producing seals (fig, 7, 8. The representation in the central field and the contents of the inscription bear witness to the fact that the seal-die belonged to Prince Lazar. A helmet with bull horns engraved on the front side is understood to be the coat of arms of Prince

  6. Lazare and Sadi Carnot a scientific and filial relationship

    CERN Document Server

    Gillispie, Charles Coulston

    2014-01-01

    Lazare Carnot was the unique example in the history of science of someone who inadvertently owed the scientific recognition he eventually achieved to earlier political prominence. He and his son Sadi produced work that derived from their training as engineers and went largely unnoticed by physicists for a generation or more, even though their respective work introduced concepts that proved fundamental when taken up later by other hands. There was, moreover, a filial as well as substantive relation between the work of father and son. Sadi applied to the functioning of heat engines the analysis that his father had developed in his study of the operation of ordinary machines. Specifically, Sadi's idea of a reversible process originated in the use his father made of geometric motions in the analysis of machines in general. This unique book shows how the two Carnots influenced each other in their work in the fields of mechanics and thermodynamics, and how future generations of scientists have further benefited f...

  7. ANCA-negative Churg-Strauss Syndrome

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    Syed Jamil Abdal

    2016-08-01

    Full Text Available A rare and a disease of unknown etiology, Churg-Strauss syndrome (CSS is a granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia, which is initially described by Churg and Strauss in 1951. Because of its clinical and pathological features that overlap with those of the other anti-neutrophil antibody (ANCA-associated systemic vasculitides (AASVs and now the disease is classified as AASVs. The ANCA status may dictate the clinical phenotype. ANCA-positive patients are significantly more likely to have disease manifesta­tions associated with small-vessel vasculitis, including oecrotising glomemlonephritis, mononeuritis and purpura, whereas ANCA-negative cases predominantly likely to have cardiac and lung involvement. The objective of this case report is to point out the possibility of vasculitic rash in ANCA-negative CSS in a 35-year-old man and the disease rarely occurs in Bangladeshi population. We analyze the history, clinical examinations and relevant investigations related to the patient to establish the diagnosis in our department. The clinical scenario and biopsy help us to attain the diagnosis. But due to unavailability of patients' cohort we have limitations of comparison of ANCA status in Bangladeshi populations. Though ANCA-positive and ANCA-negative CSS differ phenotypically, primary therapy for both the conditions is systemic glucocorticoids. Additional immunosuppressive agents like cyclophosphamide, mycophenolate mofetil, azathioprine, rituxin1ab are occasionally added in patients with more advanced or refractory disease.

  8. Interstital lung disease in ANCA vasculitis.

    Science.gov (United States)

    Alba, Marco A; Flores-Suárez, Luis Felipe; Henderson, Ashley G; Xiao, Hong; Hu, Peiqi; Nachman, Patrick H; Falk, Ronald J; Charles Jennette, J

    2017-07-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase. In the majority of these patients pulmonary fibrosis occurs concurrently or predates the diagnosis of ANCA vasculitis. More importantly, these studies have shown that ILD has an adverse impact on the long-term prognosis of ANCA vasculitis. This review focuses on the main clinical and radiologic features of pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibodies. Major histopathology features, prognosis and therapeutic options are summarized. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. THE VITA OF LAZAR OF MUROM AS A HISTORICAL SOURCE OF FYODOR GLINKA'S POEM KARELIA

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    Alexandr Mikhailovich Pashkov

    2014-11-01

    Full Text Available The article proves that one of the sources of Fyodor Glinka’s poem Karelia was the legend describing The Vita of Lazar of Murom — a Greek monk who founded the Murom Monastery of St. Assumption on the southeastern shore of Onega Lake in the middle of the 14th century. This fact has not been mentioned yet in any of the literature studies devoted to Glinka’s works. It is believed that The Vita of Lazar of Murom was created during the period between the late 14th and early 15th century. At the end of the 18th century this manuscript’s copy belonged to a high rank Petrozavodsk offi cial I. A. Pykhtin. In 1805, he consigned his copy of The Vita of Lazar of Murom to a well-known Petrozavodsk historian T. V. Balandin, who made another copy of it. Soon T.V. Balandin transferred his copy to a famous Orthodox church historian Evgenii (Bolkhovotinov, who in turn published this copy in 1813 in the 5th volume of his work The History of Russian Hierarchy. In 1826–1830, poet Fyodor Glinka was sent to exile in Petrozavodsk for having participated in the Decembrist movement. He met T. V. Balandin, read the text of The Vita of Lazar of Murom and included a story about a monk who had moved from Greece to Russia into his poem Karelia, published in 1830. Besides, the name of Saint Lazar is mentioned in the main text of the poem and twice in its footnotes. Glinka had a great intuitive feel for a language and noticed correlation between style and authenticity of the legend about Lazar’s life. So, The Vita of Lazar of Murom became one of the sources which inspired Fyodor Glinka to create his poem Karelia and to certain extent determined its content.

  10. Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance.

    Science.gov (United States)

    Mavrogeni, Sophie; Karabela, Georgia; Gialafos, Elias; Stavropoulos, Efthymios; Spiliotis, George; Katsifis, Gikas; Kolovou, Genovefa

    2013-10-01

    The cardiovascular magnetic resonance (CMR) pattern of Churg-Strauss syndrome (CSS) includes myopericarditis, diffuse subendocardial vasculitis or myocardial infarction with or without cardiac symptoms and is usually associated with lack of antineutrophil cytoplasmic antibodies (ANCA). To correlate the CMR pattern with ANCA in CSS, compare it with healthy controls and systemic lupus erythematosus (SLE) patients and re-evaluate 2 yrs after the first CMR. 28 consecutive CSS, aged 42±7 yrs, were referred for CMR and 2 yrs re-evaluation. The CMR included left ventricular ejection fraction (LVEF), T2-weighted (T2-W), early (EGE) and late gadolinium enhanced (LGE) imaging. Their results were compared with 28 systemic lupus erythematosus (SLE) under remission and 28 controls with normal myocardial perfusion, assessed by scintigraphy. CMR revealed acute cardiac lesions in all ANCA (-) CSS with active disease and acute cardiac symptoms and only in one asymptomatic ANCA (+) CSS, with active disease. Diffuse subendocardial fibrosis (DSF) or past myocarditis was identified in both ANCA(+) and ANCA (-) CSS, but with higher incidence and fibrosis amount in ANCA (-) CSS (p<0.05). In comparison to SLE, both ANCA (+) and ANCA (-) CSS had higher incidence of DSF, lower incidence of myocarditis and no evidence of myocardial infarction, due to coronary artery disease (p<0.05). In 2 yrs CMR follow up, 1/3 of CSS with DSF presented LV function deterioration and one died, although immunosuppressive treatment was given early after CSS diagnosis. Cardiac involvement either as DSF or myocarditis, can be detected in both ANCA (+) and ANCA (-) CSS, although more clinically overt in ANCA (-). DSF carries an ominous prognosis for LV function. CMR, due to its capability to detect disease severity, before cardiac dysfunction takes place, is an excellent tool for CSS risk stratification and treatment individualization.

  11. The ANCA Vasculitis Questionnaire (AAV-PRO©)

    Science.gov (United States)

    2017-05-01

    Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (Wegener's) (GPA); Wegener Granulomatosis (WG); Microscopic Polyangiitis (MPA); ANCA-Associated Vasculitis (AAV); Vasculitis

  12. Long-term patient survival in ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten

    2011-01-01

    Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.......Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain....

  13. ANCA / MPO / PR3 Antibodies Test

    Science.gov (United States)

    ... Accessed June 2010. Trevisin, M. et. al. (2008 March 10). Antigen-Specific ANCA ELISAs Have Different Sensitivities for Active and Treated Vasculitis and for Nonvasculitic Disease. Medscape from American Journal of Clinical Pathology . 2008;129(1):42-53 [On-line information]. ...

  14. Pathogenesis of ANCA-Associated Vasculitis, an Update

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2011-01-01

    Clinical observations, including a report of neonatal vasculitis occurring in a child born from a mother with anti-neutrophil cytoplasmic antibody directed to myeloperoxidase (MPO-ANCA)-associated vasculitis, suggest a pathogenic role for ANCA. Such a role is supported by in vitro experimental data

  15. Genetically distinct subsets within ANCA-associated vasculitis.

    LENUS (Irish Health Repository)

    Lyons, Paul A

    2012-07-19

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener\\'s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.

  16. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    Science.gov (United States)

    Lyons, Paul A.; Rayner, Tim F.; Trivedi, Sapna; Holle, Julia U.; Watts, Richard A.; Jayne, David R.W.; Baslund, Bo; Brenchley, Paul; Bruchfeld, Annette; Chaudhry, Afzal N.; Tervaert, Jan Willem Cohen; Deloukas, Panos; Feighery, Conleth; Gross, Wolfgang L.; Guillevin, Loic; Gunnarsson, Iva; P, Lorraine Harper M.R.C; Hrušková, Zdenka; Little, Mark A.; Martorana, Davide; Neumann, Thomas; Ohlsson, Sophie; Padmanabhan, Sandosh; Pusey, Charles D.; Salama, Alan D.; Sanders, Jan-Stephan F.; Savage, Caroline O.; Segelmark, Mårten; Stegeman, Coen A.; Tesař, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G.C.

    2013-01-01

    BACKGROUND Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis. METHODS A genomewide association study was performed in a discovery cohort of 1233 U.K. patients with ANCA-associated vasculitis and 5884 controls and was replicated in 1454 Northern European case patients and 1666 controls. Quality control, population stratification, and statistical analyses were performed according to standard criteria. RESULTS We found both major-histocompatibility-complex (MHC) and non-MHC associations with ANCA-associated vasculitis and also that granulomatosis with polyangiitis and microscopic polyangiitis were genetically distinct. The strongest genetic associations were with the antigenic specificity of ANCA, not with the clinical syndrome. Anti–proteinase 3 ANCA was associated with HLA-DP and the genes encoding α1-antitrypsin (SERPINA1) and proteinase 3 (PRTN3) (P = 6.2×10−89, P = 5.6×10−12, and P = 2.6×10−7, respectively). Anti–myeloperoxidase ANCA was associated with HLA-DQ (P = 2.1×10−8). CONCLUSIONS This study confirms that the pathogenesis of ANCA-associated vasculitis has a genetic component, shows genetic distinctions between granulomatosis with polyangiitis and microscopic polyangiitis that are associated with ANCA specificity, and suggests that the response against the autoantigen proteinase 3 is a central pathogenic feature of proteinase 3 ANCA–associated vasculitis. These data provide preliminary support for the concept that proteinase 3 ANCA–associated vasculitis and myeloperoxidase ANCA–associated vasculitis are distinct autoimmune syndromes. (Funded by the British Heart Foundation and others.) PMID

  17. Treatment of renal manifestations of ANCA-associated vasculitis.

    Science.gov (United States)

    Galesic, Kresimir; Ljubanovic, Danica; Horvatic, Ivica

    2013-01-01

    Vasculitis is a clinicopathological entity characterized by inflammation and necrosis of blood vessels. Directory of Open Access Journals (DOAJ), Google Scholar, Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched. Two major autoantigens for ANCA are myeloperoxidase (MPO) and proteinase 3 (PR3), which are proteins in the primary granules of neutrophils and in the lysosomes of monocytes. They are expressed in mature neutrophils of patients with ANCA, while absent in healthy subjects. The kidney is the most commonly affected vital organ in ANCA-associated vasculitis, and patient outcomes are largely determined by the severity of renal disease at diagnosis and by its response to treatment.

  18. Treatment of renal ANCA-associated vasculitides

    Directory of Open Access Journals (Sweden)

    Olumide Olatubosun Rowaiye

    2016-06-01

    Full Text Available Antineutrophil cytoplasmic antibody (ANCA-associated vasculitides (AAV are a group of small vessel vasculitides which commonly affect the kidneys, manifesting as rapidly progressive glomerulonephritis. In this review, we present different treatment methods (e.g. cyclophosphamide, rituximab, plasma exchange used for remission induction and maintenance in renal AAV. We also discuss treatment options in relapsing and refractory disease and for patients with end-stage renal disease due to AAV. In addition, we enumerate the various risk factors associated with relapsing and refractory disease, quality of life impairment and decreased renal and patient survival in AAV. Finally we present information on new, potentially applicable agents which can further help modify the disease course, thereby leading to increased patient survival.

  19. Genetically distinct subsets within ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Lyons, Paul A; Rayner, Tim F; Trivedi, Sapna

    2012-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single...

  20. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis

    NARCIS (Netherlands)

    Rutgers, Abraham; Slot, Marjan; van Paassen, Pieter; van Breda Vriesman, Peter; Heeringa, Peter; Tervaert, Jan Willem Cohen

    BACKGROUND: In a substantial proportion of patients with crescentic glomerulonephritis (CGN), both anti-glomerular basement membrane (GBM) antibodies and antineutrophil cytoplasmic antibodies (ANCAs) with specificity for myeloperoxidase (MPO-ANCA) are detected. In the present study, we questioned

  1. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis

    Science.gov (United States)

    ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...

  2. The Th1 and Th2 paradigm in ANCA-associated vasculitis

    NARCIS (Netherlands)

    Sanders, J S F; Stegeman, C A; Kallenberg, C G M

    2003-01-01

    In the pathogenesis of anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis, T cell contribution is indicated by T cell-dependent ANCA production combined with the presence of T cells in inflammatory infiltrates. However, the exact pathogenic role of T cells in ANCA-associated

  3. ANCA Vasculitis and Hemophagocytic Lymphohistiocytosis following a Fecal Microbiota Transplant

    Directory of Open Access Journals (Sweden)

    Adam Amlani

    2018-01-01

    Full Text Available A 69-year-old female with antisynthetase syndrome, a history of multiple recurrent infections, and documented previous negative titres for anti-neutrophil cystoplasmic antibody (ANCA suddenly developed a de novo MPO-ANCA-associated glomerulonephritis three weeks after a fecal microbiota transplantation (FMT for recurrent Clostridium difficile infections. Six months following her FMT and less than two weeks following treatment for urosepsis, she developed severe cholestasis, a markedly elevated ferritin and hypertriglyceridemia. An initial liver biopsy was suggestive of drug-induced liver injury and thus she was treated with supportive care. After she failed to improve, a second liver biopsy supported the diagnosis of hemophagocytic lymphohistiocytosis (HLH. This case highlights difficulties surrounding the early diagnosis of HLH and also questions the role of FMT and/or recurrent infections as a trigger for ANCA-associated vasculitis.

  4. [ANCA-negative subglottic laryngeal stenosis in childhood].

    Science.gov (United States)

    Wittekindt, C; Lüers, J-C; Drebber, U; Guntinas-Lichius, O; Hüttenbrink, K-B

    2007-10-01

    A 15-year-old female, having developed recurrent infections of the upper airway, hoarseness, dyspnea, and nasal congestion, was referred to our department. There was no history of trauma or intubation. The subglottic space was circularly narrowed. The test for c-ANCA was negative. Chest X-ray and renal function were normal. A tracheotomy was performed; the histology showed infiltrating plasma cells, but no signs of vasculitis or granulomatous inflammation. One year later the patient developed acute renal failure. Biopsy of the kidney confirmed Wegener's disease. The laryngeal stenosis completely resolved after therapy with cyclophosphamide. Juvenile Wegener's granulomatosis is extremely rare; the larynx and trachea seem to be involved more frequently in children than in adults. The positive testing of c-ANCA can support the diagnosis; however, even when c-ANCA do not test positive, the disease can never be excluded. Surgical interventions within the larynx or trachea might only be considered after ineffective therapy with immunosuppressive drugs.

  5. ANC A-Associated Glomerulonephritis: Relationship of main ANCA subtypes to renal outcome, age and sex of patients

    International Nuclear Information System (INIS)

    Rais-Jalali, G.; Khajehdehi, P.

    1999-01-01

    Antineutrophil cytoplasmic antibodies (ANCA) have been proven to be useful diagnostic tool in patients with systemic vasculitis with systemic vasculitis and glomerulonephritis. These antibodies exist in two types, a cytoplasmic pattern (cANCA) and a perineuclear pattern (pANCA). The effect of the main ANCA subtypes on renal outcome and its relationship to demographic findings and clinical features of patients with ANCA-associated glomerulonephritis has not been adequately studied. In this prospective study, we compared the clinical features at presentation and the renal outcome after 1 year of follow-up between two group of patients with cANCA (n=22) and pANCA (n=29) consecutively encountered over a one year period. At presentation, rapidly progressive glomerulonephritis (RPGN), and after 1 year of follow-up, end stage renal disease (ESRD) were seen more commonly in patients with pANCA than cases with cANCA (P=0.001 and P=0.04, respectively). Seropositivity for cANCA was more common in male and pANCA in female patients (P=0.05). Occurrence of the pulmonary-renal syndrome or extra-renal manifestations, such as sinusitis and skin rash, did not differ significantly among the two groups of patients with cANCA and pANCA. Patients with pANCA present more frequently with RPGN, leading to a poorer renal survival compared to cases with cANCA. RPGN and pANCA are more common in females. (author)

  6. Anca associated vasculitis : occurrence, prediction, prevention, and outcome of relapses

    NARCIS (Netherlands)

    Boomsma, Maarten Michiel

    2001-01-01

    During follow-up, relapses of disease activity occur in the majority of patients with ANCA associated vasculitis. The general objective brought together in this thesis was to further elucidate the characteristics and consequences of these relapses. Investigated items are the occurrence, the

  7. New advances in the pathogenesis of ANCA-associated vasculitides

    NARCIS (Netherlands)

    Chen, M.; Kallenberg, C. G. M.

    2009-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of autoimmune disorders including Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and renal-limited vasculitis (RLV). This paper reviews updated information on the

  8. Sacrifice and self-sacrifice of Prince Lazar in the national epic from Christian and pagan aspects

    Directory of Open Access Journals (Sweden)

    Bojanić-Ćirković Mirjana D.

    2014-01-01

    Full Text Available This paper approaches the motives of sacrifice and self-sacrifice of Prince Lazar as a key to the construction of this epic character. At the beginning, consulting dictionaries and previous research, we determine the terminology and concepts of sacrifice self-sacrifice. Approaching the analysis of these motifs in the epic poems and accepting attitudes about their developmental line that ranges from medieval to folk literature, first search for Christian self-sacrifice. In the final part of the paper we provide a synthesis of Christian and pagan influence on the shaping of Lazarus motif of sacrifice and self-sacrifice with an emphasis on their share, hierarchy and function.

  9. pANCA-vasculitis associated with rectal adenocarcinoma.

    Science.gov (United States)

    Hommel, C; Rihova, Z; Mokaddem, F; Libotte, B

    2014-12-01

    We report the case of a 69-year-old male patient who was admitted for fever, dry cough, recurrent sinusitis with epistaxis, anorexia with weight loss of 20 kg over a 3-month period, myalgia, and mononeuritis multiplex. He was diagnosed with pANCA/anti-MPO associated vasculitis and rectal adenocarcinoma. The tumor was treated by surgical resection. Recurrence of vasculitis occurred during steroid tapering which prompted us to add Mycophenolate mofetyl. A complete remission was achieved. We conclude that in the present case the vasculitis was an independent disease, not a paraneoplastic phenomenon. We discuss the value of different ANCA serologies for diagnostics and follow-up, the epidemiology of vasculitis associated with malignancy, and the concept of vasculitis as a paraneoplastic syndrome.

  10. ANCA: Anharmonic Conformational Analysis of Biomolecular Simulations.

    Science.gov (United States)

    Parvatikar, Akash; Vacaliuc, Gabriel S; Ramanathan, Arvind; Chennubhotla, S Chakra

    2018-05-08

    Anharmonicity in time-dependent conformational fluctuations is noted to be a key feature of functional dynamics of biomolecules. Although anharmonic events are rare, long-timescale (μs-ms and beyond) simulations facilitate probing of such events. We have previously developed quasi-anharmonic analysis to resolve higher-order spatial correlations and characterize anharmonicity in biomolecular simulations. In this article, we have extended this toolbox to resolve higher-order temporal correlations and built a scalable Python package called anharmonic conformational analysis (ANCA). ANCA has modules to: 1) measure anharmonicity in the form of higher-order statistics and its variation as a function of time, 2) output a storyboard representation of the simulations to identify key anharmonic conformational events, and 3) identify putative anharmonic conformational substates and visualization of transitions between these substates. Copyright © 2018 Biophysical Society. Published by Elsevier Inc. All rights reserved.

  11. Anti-GBM disease and ANCA during dengue infection.

    Science.gov (United States)

    Lizarraga, Karlo J; Florindez, Jorge A; Daftarian, Pirouz; Andrews, David M; Ortega, Luis M; Mendoza, Jair Munoz; Contreras, Gabriel N; Nayer, Ali

    2015-02-01

    Anti-glomerular basement membrane (GBM) disease is a severe inflammatory renal disorder due to pathogenic autoantibodies directed mainly against the α3 chain of type IV collagen. In ~1/4 of patients with anti-GBM disease, antineutrophil cytoplasmic antibodies (ANCA) predominantly with myeloperoxidase (MPO) specificity can be detected. Although the inciting stimuli leading to the development of an immune response against the type IV collagen and neutrophils are unknown, evidence indicates that both genetic and environmental factors play a role. Of note, molecular mimicry between self-antigens and nonself-antigens such as antigenic determinants of microorganisms has been implicated in the pathogenesis of anti-GBM disease and ANCA-associated vasculitis. A mosquito-borne viral illness highly prevalent in the tropics and subtropics, dengue can be complicated by acute renal failure, proteinuria, hematuria and glomerulonephritis. We present a 66-year-old woman who was diagnosed with dengue infection and rapidly progressive glomerulonephritis during an outbreak of dengue in Honduras in the summer of 2013. Renal biopsy revealed severe crescentic glomerulonephritis. Immunofluorescence examination demonstrated strong linear IgG deposition along glomerular capillary walls. Serologic tests demonstrated antibodies against GBM, MPO and platelet glycoproteins. The patient was diagnosed with anti-GBM disease associated with p-ANCA with MPO specificity. Despite heavy immunosuppression and plasmapheresis, IgG titers against dengue virus continued to rise confirming the diagnosis of acute dengue infection. We present the first reported case of anti-GBM disease associated with p-ANCA with MPO specificity during dengue infection. This report calls for a heightened awareness of autoimmunity leading to crescentic glomerulonephritis in patients with dengue infection.

  12. ANCA-negative limited Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Ghosh A

    2004-03-01

    Full Text Available A 26-year-old man presented with epistaxis, nasal obstruction and a subcutaneous swelling over the left malar region with radiological evidence of a mass in the right nasal cavity. Histology of the lesions showed necrotizing granuloma with evidence of vasculitis. There was no other systemic involvement and the patient was ANCA-negative. Excellent response to systemic steroid and cyclophosphamide therapy was noted.

  13. Diversity of PR3-ANCA epitope specificity in Wegener's granulomatosis. Analysis using the biosensor technology

    NARCIS (Netherlands)

    Rarok, Agnieszka; van der Geld, Y.M.; Stegeman, Coen; Limburg, Piet; Kallenberg, Cees

    Wegener's granulomatosis is a systemic disease characterized by the presence of antineutrophil cytoplasm autoantibodies specific for proteinase 3 (PR3-ANCA). The functional characteristics of PR3-ANCA differ between quiescent and active disease, suggesting changes in the properties of the

  14. C5a Receptor (CD88) Blockade Protects against MPO-ANCA GN

    OpenAIRE

    Xiao, Hong; Dairaghi, Daniel J.; Powers, Jay P.; Ertl, Linda S.; Baumgart, Trageen; Wang, Yu; Seitz, Lisa C.; Penfold, Mark E.T.; Gan, Lin; Hu, Peiqi; Lu, Bao; Gerard, Norma P.; Gerard, Craig; Schall, Thomas J.; Jaen, Juan C.

    2013-01-01

    Necrotizing and crescentic GN (NCGN) with a paucity of glomerular immunoglobulin deposits is associated with ANCA. The most common ANCA target antigens are myeloperoxidase (MPO) and proteinase 3. In a manner that requires activation of the alternative complement pathway, passive transfer of antibodies to mouse MPO (anti-MPO) induces a mouse model of ANCA NCGN that closely mimics human disease. Here, we confirm the importance of C5aR/CD88 in the mediation of anti-MPO–induced NCGN and report th...

  15. [From Algebra as art to School Algebra : The textbooks written by Pierre de La Ramée, Bernard Salignac and Lazare Schöner].

    Science.gov (United States)

    Loget, François

    2011-01-01

    Bernard Salignac and Lazare Schöner both published a treatise based on La Ramée's Algebra. Why did they endeavour to republish a book that their master had abandoned ? What changes did they make in the original ? They turned algebra into apedagogical discipline at a time when Ramism was making its presence felt in some Northern European educational establishments. Salignac took La Ramée's thoughts concerning the language of mathematics further and offered an original and precocious contribution to what I call the "scriptural turn" of mathematics at the end of the 16(th) century.

  16. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    Directory of Open Access Journals (Sweden)

    Kimberly P. Liang

    2011-01-01

    Full Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse; ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43% died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

  17. Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis.

    Science.gov (United States)

    Yoshida, Naohiro; Iino, Yukiko

    2014-12-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is histologically characterized by systemic necrotizing vasculitis and is clinically classified into two phases, systemic or localized. Recently, otological symptoms such as otitis media and hearing loss, not previously often associated with AAV, have been reported in AAV cases. In these cases we propose a diagnosis of otitis media with AAV (OMAAV). The ANCA titer is important for the diagnosis of OMAAV, and in most cases rapid progressive hearing loss is observed as localized AAV. Peripheral facial nerve palsy or hypertrophic pachymeningitis are coupled with 25% of cases and 18% of cases respectively. Proteinase 3-ANCA (PR3-ANCA) positive otitis media causes granulomatous formation or middle ear effusion in the middle ear, on the other hand myeloperoxidase-ANCA (MPO-ANCA) positive otitis media predominantly presents as otitis media with effusion. The early diagnosed case and the sensorineural hearing loss not progressed deaf could be recovered by the immunosuppressive therapy. Delayed diagnosis of AAV occasionally leads to progression to the irreversible phase; therefore, diagnosis at the early-localized stage is important for treating AAV. In this review, we discuss the current understanding of this newly proposed concept of OMAAV.

  18. [Rapidly progressive ANCA positive glomerulonephritis as the presenting feature of infectious endocarditis].

    Science.gov (United States)

    Hanf, W; Serre, J-E; Salmon, J-H; Fabien, N; Ginon, I; Dijoud, F; Trolliet, P

    2011-12-01

    The association of positive cytoplasmic antineutrophil antibody (ANCA) necrotizing crescentic glomerulonephritis with endocarditis raises diagnostic issues. Indeed, it is often difficult to determine if the kidney injury is either secondary to an infectious disease or caused by an ANCA-associated small vessel vasculitis. We report a 59-year-old man admitted in nephrology for acute glomerular syndrome in whom the renal biopsy showed a crescentic necrotizing glomerulonephritis. A diagnosis of vasculitis was initially considered in the presence of high titer of ANCA (anti-proteinase 3). Because of associated Staphyloccocus aureus endocarditis the patient received both corticosteroids and antibiotics that allowed remission of both kidney injury and endocarditis. The renal presentation and the disappearance of ANCA support the infectious etiology of this glomerulonephritis rather than an ANCA-associated small vessel vasculitis. It is important to be cautious in the presence of ANCA positive extracapillary glomerulonephritis and endocarditis should be ruled out before initiation of corticosteroids that may be nevertheless necessary in severe acute glomerulonephritis. Copyright © 2011 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  19. ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma

    OpenAIRE

    Cervi, Andrea; Kelly, Dylan; Alexopoulou, Iakovina; Khalidi, Nader

    2017-01-01

    Purpose: We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. Methods: On systematic review of the literature, we found five previous cases...

  20. Diagnostic accuracy of atypical p-ANCA in autoimmune hepatitis using ROC- and multivariate regression analysis.

    Science.gov (United States)

    Terjung, B; Bogsch, F; Klein, R; Söhne, J; Reichel, C; Wasmuth, J-C; Beuers, U; Sauerbruch, T; Spengler, U

    2004-09-29

    Antineutrophil cytoplasmic antibodies (atypical p-ANCA) are detected at high prevalence in sera from patients with autoimmune hepatitis (AIH), but their diagnostic relevance for AIH has not been systematically evaluated so far. Here, we studied sera from 357 patients with autoimmune (autoimmune hepatitis n=175, primary sclerosing cholangitis (PSC) n=35, primary biliary cirrhosis n=45), non-autoimmune chronic liver disease (alcoholic liver cirrhosis n=62; chronic hepatitis C virus infection (HCV) n=21) or healthy controls (n=19) for the presence of various non-organ specific autoantibodies. Atypical p-ANCA, antinuclear antibodies (ANA), antibodies against smooth muscles (SMA), antibodies against liver/kidney microsomes (anti-Lkm1) and antimitochondrial antibodies (AMA) were detected by indirect immunofluorescence microscopy, antibodies against the M2 antigen (anti-M2), antibodies against soluble liver antigen (anti-SLA/LP) and anti-Lkm1 by using enzyme linked immunosorbent assays. To define the diagnostic precision of the autoantibodies, results of autoantibody testing were analyzed by receiver operating characteristics (ROC) and forward conditional logistic regression analysis. Atypical p-ANCA were detected at high prevalence in sera from patients with AIH (81%) and PSC (94%). ROC- and logistic regression analysis revealed atypical p-ANCA and SMA, but not ANA as significant diagnostic seromarkers for AIH (atypical p-ANCA: AUC 0.754+/-0.026, odds ratio [OR] 3.4; SMA: 0.652+/-0.028, OR 4.1). Atypical p-ANCA also emerged as the only diagnostically relevant seromarker for PSC (AUC 0.690+/-0.04, OR 3.4). None of the tested antibodies yielded a significant diagnostic accuracy for patients with alcoholic liver cirrhosis, HCV or healthy controls. Atypical p-ANCA along with SMA represent a seromarker with high diagnostic accuracy for AIH and should be explicitly considered in a revised version of the diagnostic score for AIH.

  1. Clinical features and outcomes of ANCA-associated renal vasculitis

    Directory of Open Access Journals (Sweden)

    Sidy Mohamed Seck

    2012-01-01

    Full Text Available To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG, seven as microscopic polyangitis (MPA and one as Churg-Strauss syndrome (SCS. The median age of the patients was 57.7 years and the sex-ratio (M/F was 1.6. The visceral mani-festations included kidneys (100% of patients, lungs (75%, ENT (52% of WG, and nervous system (57% of MPA. The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28% compared to WG cases (12%. After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up.

  2. Innere Emigration und Diktatur die getarnten Botschaften in den Opern der meister aus bojana von Konstantin Iliev und der gefesselte Prometeus von Lazar Nikolov

    Directory of Open Access Journals (Sweden)

    Kostakeva Maria

    2004-01-01

    Full Text Available (nemački Die Frage der "inneren Emigration" in den ehemaligen kommunistischen Ländern Europas wird an Beispielen zweier Opern der wichtigen bulgarischen Komponisten Konstantin Iliev und Lazar Nikolov untersucht. Ähnlich wie Paul Hindemith in Mathis der Maler, in Ilievs Meister aus Bojana (1962, kam die Inspiration von einer mittelalterlichen Legende her, und iM Mittelpunkt des Dramas steht das Problem der Freiheit der Schöpfer unter der dogmatischen Gewalt. IM Gefesselten Prometeus (1972 von Nikolov ist die Hauptperson als Symbol des Protests gegen die Tyrannei und die Unterdrückung zu verstehen und so lässt sich in allen Zeiten politisch interpretieren, umso mehr bei einem kommunistischen Regime.

  3. Avallon (Yonne, note sur l’effondrement du petit surplomb du rempart, au chevet de la collégiale Saint-Lazare

    Directory of Open Access Journals (Sweden)

    Virginie Jolly

    2006-09-01

    Full Text Available Suite à l’effondrement d’un élément de l’enceinte urbaine durant l’hiver 2004, une intervention a été souhaitée conjointement par le Service Régional de l’Archéologie de Dijon (DRAC Bourgogne et la Municipalité d’Avallon (Yonne. La zone concernée, située à l’est de la ville médiévale, sur le rempart, est en contact direct avec l’esplanade du chevet de la collégiale Saint-Lazare. La configuration escarpée et dangereuse du terrain —instabilité du sol en partie haute et effritement du mur— a l...

  4. Proinflammatory genotype of interleukin-1 and interleukin-1 receptor antagonist is associated with ESRD in proteinase 3-ANCA vasculitis patients.

    Science.gov (United States)

    Borgmann, Stefan; Endisch, Georg; Hacker, Ulrich T; Song, Bong-Seok; Fricke, Harald

    2003-05-01

    Small-vessel vasculitides are associated with antineutrophil cytoplasmic antibodies (ANCAs). Cytoplasmic ANCAs are targeted mainly against proteinase 3 (PR3), whereas myeloperoxidase (MPO) is the major antigen of perinuclear ANCAs. These relapsing vasculitides show heterogeneous clinical pictures, and disease severity may vary broadly from mild local organ manifestation to acute organ failure (eg, renal failure). We tested whether two cytokine polymorphisms in the interleukin-1beta (IL-1beta) and IL-1 receptor antagonist (IL-1ra) genes, known to determine cytokine secretion, are associated with clinical manifestations and outcome of ANCA-associated vasculitides. Polymerase chain reaction and restriction fragment length polymorphism analyses were performed to determine polymorphisms in the IL-1beta and IL-1ra genes in 79 patients with PR3-ANCA, 30 patients with MPO-ANCA vasculitis, and 196 healthy controls. The frequency of the so-called proinflammatory genotype, characterized by high secretion of IL-1beta and low secretion of its antagonist IL-1ra, was increased significantly in patients with PR3-ANCA with end-stage renal disease. Patients with a renal manifestation of PR3-ANCA vasculitis have an increased risk for developing end-stage renal disease when carrying the proinflammatory IL-1beta/IL-1ra genotype. Anti-inflammatory therapy specifically antagonizing the proinflammatory effect of IL-1beta may be a promising treatment for patients with Wegener's granulomatosis with renal manifestations.

  5. Perinuclear anti-neutrophil cytoplasmic antibodies (p-anca) in chronic ulcerative colitis: Experience in a Mexican institution

    Science.gov (United States)

    Yamamoto-Furusho, Jesus K; Takahashi-Monroy, Takeshi; Vergara-Fernandez, Omar; Reyes, Edgardo; Uscanga, Luis

    2006-01-01

    AIM: To assess the prevalence and clinical value of p-ANCA in a sample of Mexican ulcerative colitis (UC) patients. METHODS: In a prospective, IRB-approved protocol, p-ANCA was determined in 80 patients with UC (mean age, 32 ± 12.9 years). The severity and extension of disease were determined by clinical methods, searching a statistical association with p-ANCA status. RESULTS: p-ANCA were detected in 41 (51%) patients. Severity of disease was the only clinical variable statistically associated with their presence (P < 0.0001; OR = 9; CI 95% = 3.2-24.7). CONCLUSION: The prevalence of p-ANCA was similar to that reported in other countries. Their presence was associated to UC severity, but offered no more information than the obtained by clinical methods. PMID:16733859

  6. Epitope analysis of anti-myeloperoxidase antibodies in patients with ANCA-associated vasculitis.

    Directory of Open Access Journals (Sweden)

    Shen-Ju Gou

    Full Text Available OBJECTIVE: Increasing evidences have suggested the pathogenic role of anti-neutrophil cytoplasmic antibodies (ANCA directing myeloperoxidase (MPO in ANCA-associated vasculitis (AAV. The current study aimed to analyze the association between the linear epitopes of MPO-ANCA and clinicopathological features of patients with AAV. METHODS: Six recombinant linear fragments, covering the whole length amino acid sequence of a single chain of MPO, were produced from E.coli. Sera from 77 patients with AAV were collected at presentation. 13 out of the 77 patients had co-existence of serum anti-GBM antibodies. Ten patients also had sequential sera during follow up. The epitope specificities were detected by enzyme-linked immunosorbent assay using the recombinant fragments as solid phase ligands. RESULTS: Sera from 45 of the 77 (58.4% patients with AAV showed a positive reaction to one or more linear fragments of the MPO chain. The Birmingham Vasculitis Activity Scores and the sera creatinine were significantly higher in patients with positive binding to the light chain fragment than that in patients without the binding. The epitopes recognized by MPO-ANCA from patients with co-existence of serum anti-GBM antibodies were mainly located in the N-terminus of the heavy chain. In 5 out of the 6 patients, whose sera in relapse recognize linear fragments, the reactivity to linear fragments in relapse was similar to that of initial onset. CONCLUSION: The epitope specificities of MPO-ANCA were associated with disease activity and some clinicopathological features in patients with ANCA-associated vasculitis.

  7. ANCA-associated pauci-immune glomerulonephritis in a patient with bacterial endocarditis: a challenging clinical dilemma.

    Science.gov (United States)

    Cervi, Andrea; Kelly, Dylan; Alexopoulou, Iakovina; Khalidi, Nader

    2017-01-01

    We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. On systematic review of the literature, we found five previous cases whereby IE caused by streptococcus and bartonella species were related to ANCA vasculitis-associated GN. Most reports of IE-related GN are mediated by immune complex deposition and resolve following microbial clearance. Of the 5 cases of ANCA GN in the setting of IE, all had markedly elevated levels of PR3-ANCA with either a subacute or chronic course of infection. Patients were treated with a combination of steroids and cyclophosphamide (2/5), steroids and antibiotics alone (1/5), or with valvular replacement (2/5). Renal function was recovered in 4/5 patients. Infection is a major etiologic player in the formation of ANCA; however, the role of PR3-ANCA in IE remains unclear. Kidney biopsy is essential in differentiating IE-related GN due to infection and immune complex deposition versus ANCA-associated vasculitis. A paucity of reports on the development of GN in IE-associated ANCA vasculitis exists, highlighting the rarity of our case and lack of clear therapeutic strategies in a patient with active infection requiring immunosuppression. In this case, the patient's chronic hepatitis B and C coinfection presented a unique challenge.

  8. Recurrence of ANCA-associated vasculitis in a patient with kidney trasplant

    Directory of Open Access Journals (Sweden)

    Pedro García Cosmes

    2016-03-01

    Full Text Available Renal disease secondary to vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA can lead to chronic renal disease requiring renal replacement therapy. In these patients, kidney transplantation offers excellent long-term rates of allograft and patient survival; consequently, they can be trasplanted when the clinical disease activity has remitted. However, the risk of disease relapses in the renal allograft remains, although at lower rates due to modern immunosuppressive regimes. We describe the case of a male patient with extracapillary glomerulonephritis type III C-ANCA (+ who developed a recurrence in the renal allograft 8 years after transplantation. Intensive immunosupression with plasmapheresis controlled the disease.

  9. The idea of the perfect city in the state of prince Lazar and despotes Stefan Lazarević

    Directory of Open Access Journals (Sweden)

    Radojčić Svetozar

    2008-01-01

    foundations. The new wave of piety originated from the circles of educated refugee monks, who found refuge in the small palaces, where a rather complex, monastic-feudal mentality gradually formed. The palace culture of those centers had their own pious literature, where various visions of the invisible Paradise were particularly widely read. The old idea that all the righteous would attain Heaven gained a new importance and a new interpretation at the end of the XIV century. This faith in the habitation of Heaven began to be linked to buildings on earth - to cities and monasteries. The depiction of Heaven, or the Upper Jerusalem, as it was called, had already begun in Serbian painting in the mid-fourteenth century. Around 1400, however, there was an insistence on presenting how the organization of the palace was similar to the heavenly hierarchy, how the courtiers with their appearance looked like the inhabitants of Paradise, and how the building the royal court erected resembled the heavenly houses of the 'New Jerusalem'. Such descriptions about the resemblance to the dwellings of Heaven were very frequent in Serbian literature of the late fourteenth and early fifteenth centuries. Prince Lazar resorted with increasing frequency to creating buildings according to the image of the 'Upper Jerusalem', especially the monastery of Ravanica. Andonije Epaktit gave the most exhaustive account of Ravanica-Jerusalem: Ravanica was the 'dwelling of God' and the 'door to Heaven', it had seven pinnacles or pyrgoi, and accordingly, it was similar to Jerusalem, Rome and Constantinople, and it was inhabited by monk-angels. Constantme the Philosopher wrote in a similar vein. His magnificent description of Belgrade as Jerusalem in the Life of Despotes Stefan Lazarević falls among the most important eulogies of the cities in Byzantine culture. And Belgrade - like Jerusalem, Rome and Constantinople - has 'seven pinnacles'. It resembles both the 'lower' and the 'higher' Jerusalem; so as to

  10. Intermediate monocytes in ANCA vasculitis: increased surface expression of ANCA autoantigens and IL-1β secretion in response to anti-MPO antibodies.

    LENUS (Irish Health Repository)

    O'Brien, Eóin C

    2015-01-01

    ANCA vasculitis encompasses several autoimmune conditions characterised by destruction of small vessels, inflammation of the respiratory tract and glomerulonephritis. Most patients harbour autoantibodies to myeloperoxidase (MPO) or proteinase 3 (PR3). Clinical and experimental data suggest that pathogenesis is driven by ANCA-mediated activation of neutrophils and monocytes. We investigated a potential role for distinct monocyte subsets. We found that the relative proportion of intermediate monocytes is increased in patients versus control individuals, and both MPO and PR3 are preferentially expressed on these cells. We demonstrate that MPO and PR3 are expressed independently of each other on monocytes and that PR3 is not associated with CD177. MPO expression correlates with that of Fc receptor CD16 on intermediate monocytes. Monocyte subsets respond differently to antibodies directed against MPO and PR3, with anti-MPO but not anti-PR3 leading to increased IL-1β, IL-6 and IL-8 production. In concordance with the observed higher surface expression of MPO on intermediate monocytes, this subset produces the highest quantity of IL-1β in response to anti-MPO stimulation. These data suggest that monocytes, specifically, the intermediate subset, may play a role in ANCA vasculitis, and also indicate that substantial differences exist between the effect of anti-MPO and anti-PR3 antibodies on these cells.

  11. Mechanisms of vasculitis : How pauci-immune is ANCA-associated renal vasculitis?

    NARCIS (Netherlands)

    van Paassen, P.; Tervaert, J. W. Cohen; Heeringa, P.

    2007-01-01

    Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis. However, anti-neutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis is characterized by a 'pauci-immune' pattern of immunofluorescence during kidney biopsy, indicating the relative

  12. North Country Successes: Case Studies of Successful Entrepreneurs in the ANCA Region.

    Science.gov (United States)

    Chugh, Ram L.; Gandhi, Prem P.

    This study identifies the characteristics of both successful small businesses and their entrepreneurial owners in a 14-county area of the Adirondack North Country Association (ANCA). Of the 100 survey respondents representing successful small businesses, 50% had been in business for less than 14 years; 38% were in manufacturing; 48% employed more…

  13. Genetic loci of Staphylococcus aureus associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides

    NARCIS (Netherlands)

    Glasner, Corinna; de Goffau, Marcus C; van Timmeren, Mirjan M; Schulze, Mirja L; Jansen, Benita; Tavakol, Mehri; van Wamel, Willem J B; Stegeman, Coen A; Kallenberg, Cees G M; Arends, Jan P; Rossen, John W; Heeringa, Peter; van Dijl, Jan Maarten

    2017-01-01

    The proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) granulomatosis with polyangiitis (GPA) has been associated with chronic nasal S. aureus carriage, which is a risk factor for disease relapse. The present study was aimed at comparing the

  14. Genetic loci of Staphylococcus aureus associated with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides

    NARCIS (Netherlands)

    C. Glasner (Corinna); M.C. De Goffau (Marcus C.); M.M. Van Timmeren (Mirjan M.); Schulze, M.L. (Mirja L.); Jansen, B. (Benita); M. Tavakol (Mehri); W.J.B. van Wamel (Willem); C.A. Stegeman; C.G.M. Kallenberg (Cees G. M.); J.P.A. Arends (Jan); J.W. Rossen (John); P. Heeringa (Peter); J.M. Dijl (Jan Maarten)

    2017-01-01

    textabstractThe proteinase 3 (PR3)-positive anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) granulomatosis with polyangiitis (GPA) has been associated with chronic nasal S. aureus carriage, which is a risk factor for disease relapse. The present study was aimed at

  15. C5a receptor (CD88) blockade protects against MPO-ANCA GN.

    Science.gov (United States)

    Xiao, Hong; Dairaghi, Daniel J; Powers, Jay P; Ertl, Linda S; Baumgart, Trageen; Wang, Yu; Seitz, Lisa C; Penfold, Mark E T; Gan, Lin; Hu, Peiqi; Lu, Bao; Gerard, Norma P; Gerard, Craig; Schall, Thomas J; Jaen, Juan C; Falk, Ronald J; Jennette, J Charles

    2014-02-01

    Necrotizing and crescentic GN (NCGN) with a paucity of glomerular immunoglobulin deposits is associated with ANCA. The most common ANCA target antigens are myeloperoxidase (MPO) and proteinase 3. In a manner that requires activation of the alternative complement pathway, passive transfer of antibodies to mouse MPO (anti-MPO) induces a mouse model of ANCA NCGN that closely mimics human disease. Here, we confirm the importance of C5aR/CD88 in the mediation of anti-MPO-induced NCGN and report that C6 is not required. We further demonstrate that deficiency of C5a-like receptor (C5L2) has the reverse effect of C5aR/CD88 deficiency and results in more severe disease, indicating that C5aR/CD88 engagement enhances inflammation and C5L2 engagement suppresses inflammation. Oral administration of CCX168, a small molecule antagonist of human C5aR/CD88, ameliorated anti-MPO-induced NCGN in mice expressing human C5aR/CD88. These observations suggest that blockade of C5aR/CD88 might have therapeutic benefit in patients with ANCA-associated vasculitis and GN.

  16. Patient perceptions about illness self-management in ANCA-associated small vessel vasculitis.

    Science.gov (United States)

    Thorpe, C T; DeVellis, R F; Blalock, S J; Hogan, S L; Lewis, M A; DeVellis, B M

    2008-06-01

    To characterize patient perceptions, related to eight self-management behaviours relevant for adults with ANCA-associated small vessel vasculitis (ANCA-SVV), and to determine if these perceptions were associated with performance of each behaviour. Adults with ANCA-SVV (n = 202) completed a self-administered questionnaire that assessed eight self-management behaviours (adherence to recommendations for medication, health service use, diet, exercise, infection avoidance and symptom monitoring; prompt reporting of symptoms and side effects; and adjusting activities in response to symptoms), perceptions about these behaviours, socio-demographics, clinical factors and social desirability bias. Descriptive statistics were generated to characterize patients' perceptions about difficulty of, importance of, and specific barriers to performing each behaviour. Regression analyses explored whether these variables were associated with performing each behaviour, controlling for potential confounders. With few exceptions, higher perceived importance and lower perceived difficulty of each behaviour were associated with more frequent performance of the behaviour. For each behaviour, several specific barriers were frequently endorsed by patients and a number of these were associated with lower levels of self-management. This study reveals that patient perceptions about the illness and its treatment influence ANCA-SVV self-management. Perceived barriers to medication, health services, diet and exercise adherence were similar to those in other illnesses. This study also provides insight into barriers experienced by patients in performing behaviours (infection avoidance, symptom monitoring, reporting symptoms and side-effects and adjusting activities) not often previously studied. How the identification of these barriers can help inform future interventions for ANCA-SVV patients is to be discussed.

  17. Performance of two strategies for urgent ANCA and anti-GBM analysis in vasculitis.

    Science.gov (United States)

    de Joode, Anoek A E; Roozendaal, Caroline; van der Leij, Marcel J; Bungener, Laura B; Sanders, Jan Stephan F; Stegeman, Coen A

    2014-02-01

    In anti-neutrophil cytoplasmic antibodies (ANCA) associated small vessel vasculitis (AAV), rapid testing for ANCA and anti-glomerular basement membrane (GBM) antibodies may be beneficial for therapeutic purpose. We analysed the diagnostic performance of two rapid ANCA and anti-GBM test methods in 260 patients with suspected AAV. Between January 2004 and November 2010, we analysed 260 samples by qualitative Dotblot (Biomedical Diagnostics); retrospective analysis followed with directly coated highly sensitive automated Phadia ELiA and ELiA anti-GBM. Results were related to the final clinical diagnosis and compared with routine capture ELISA. Seventy-four patients had a final diagnosis of AAV (n=62) or anti-GBM disease (n=12). Both Dotblot and ELiA detected all 12 cases of anti-GBM disease; 2 false positive results were found. Dotblot detected ANCA in 56 of 62 AAV patients (sensitivity 90%, NPV 97%), and showed 5 false positives (specificity 97%, PPV 90%). The Phadia ELiA anti-PR3(s) or anti-MPO(s) was positive in 57 of 62 AAV patients (sensitivity 92%, NPV 97%), and had 5 false positives (specificity 97%, PPV 88%). Routine capture ELISA was equally accurate (sensitivity 94%, specificity 97%, PPV 88%, NPV 98%). The Dotblot and Phadia ELiA on anti-GBM, anti-PR3(s) and anti-MPO(s) performed excellently; results were almost identical to routine ELISA. When suspicion of AAV or anti-GBM disease is high and diagnosis is urgently needed, both tests are very powerful for rapid serological diagnosis. Further studies have to confirm the test performances in samples routinely presented for ANCA testing and in follow-up of positive patients. Copyright © 2013 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

  18. Plasmapheresis Rescue Therapy in Progressive Systemic ANCA-Associated Vasculitis : Single-Center Results of Stepwise Escalation of Immunosuppression

    NARCIS (Netherlands)

    de Joode, Anoek A. E.; Sanders, Jan Stephan; Smid, W. Martin; Stegeman, Coen A.

    2014-01-01

    Objective: We evaluated 26 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) with progressive disease despite treatment with cyclophosphamide and steroids treated with additional plasmapheresis and compared outcome with 50 matched-disease controls. Methods:

  19. [Cavitating lung lesions in the course of ANCA-associated vasculitis: differential diagnostic aspects].

    Science.gov (United States)

    Kirchner, J; Raab, H P; Länger, F; Wigand, R; Mitrou, P; Jacobi, V

    1998-05-01

    Antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitides (Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome) show quite variable courses. Clinical features of the full blown generalized systemic vasculitis are usually found in the respiratory tract and the kidney. Pulmonary involvement of Wegener's granulomatosis shows commonly nodules and cavitations but also diffuse alveolar hemorrhage. We report the case of a 57 year-old man suffering from dyspnea, thoracal pain, arthralgia, purpura, scleritis and tinitus. Specimen of the kidney showed segmental glomerulosclerosis and tubulointerstitial nephritis. Because of the presence of cANCA Wegener's disease was assumed. Pulmonary infiltrates developed under immunosuppressive treatment with cyclophosphamid. As differential diagnosis of the pulmonary infiltrates, we considered invasive pulmonary aspergillosis as well as infiltrates due to Wegener's granulomatosis. In spite of maximal therapeutic management of patient died of respiratory and cardiovascular failure. The findings at autopsy showed distinct invasive pulmonary aspergillosis and perifocal hemorrhage.

  20. ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.

    Science.gov (United States)

    Psychogios, Klearchos; Evmorfiadis, Ilias; Dragomanovits, Spyros; Stavridis, Athanasios; Takis, Konstantinos; Kaklamanis, Loukas; Stathis, Pantelis

    2017-03-01

    Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA. This case contributes to the recent debate of the 2 possible presentations of the disease according to the ANCA (antineutrophil cytoplasmic antibodies) status. We furthermore underscore the need for careful differential diagnosis of the "ANCA negative" cases with persistent hypereosinophilia from the idiopathic hypereosinophilic syndrome. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  1. Data of evolutionary structure change: 1ANCA-2HNTE [Confc[Archive

    Lifescience Database Archive (English)

    Full Text Available 1ANCA-2HNTE 1ANC 2HNT A E IVGGYTCQENSVPYQVSLNSGYHFCGGSLINDQWVVSAA...> 0 1ANC A 1ANC...1 1.00 16.75 C e-map> ILE ASP ASN ASN LEU THR LYS ARG ASP PHE ASN CA 5.65 3.82 ASP CA 3.82 ILE CA ...21.33 C e-map> ILE ASP ARG ASP LEU ASN

  2. Central retinal artery occlusion in a patient with ANCA-negative Churg-Strauss syndrome

    Science.gov (United States)

    Kumano, Yuji; Yoshida, Noriko; Fukuyama, Satoru; Miyazaki, Masanori; Enaida, Hiroshi; Matsui, Takaaki

    2012-01-01

    Ocular involvement in Churg-Strauss syndrome is infrequent. We describe the case of a 54-year-old woman with eosinophilia and involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for Churg-Strauss syndrome. The patient presented with acute, painless vision loss in her right eye. Central retinal artery occlusion (CRAO) without accompanying retinal vasculitis was diagnosed by angiographic findings and funduscopic findings of retinal whitening with a cherry-red spot. Although her antineutrophil cytoplasmic antibody (ANCA) status was negative, CRAO was thought to be an ocular manifestation of Churg-Strauss syndrome, and appropriate treatment was planned. She was treated with high-dose corticosteroids and anticoagulant therapy. Her macular edema improved, but visual recovery was poor. Specific therapy to alter inflammation, blood coagulation, and rheology reportedly plays an important role in ANCA-positive patients with Churg-Strauss syndrome who develop CRAO. Regardless of ANCA status, high-dose corticosteroids should be considered for CRAO in patients with Churg-Strauss syndrome, as discussed in this case. PMID:22927731

  3. Paquimeningitis hipertrófica, glomerulonefritis y vasculitis de pequeños vasos asociada a ANCA Hypertrophic pachymeningitis, glomerulonephritis and P-ANCA associated small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Octavio Mazzocchi

    2007-04-01

    Full Text Available La paquimeningitis hipertrófica es una enfermedad poco frecuente caracterizada por engrosamiento de la duramadre. Presentamos una paciente con esta enfermedad que se manifestó con cefalea crónica y en la que concomitantemente se evidenció una glomerulonefritis necrotizante extracapilar pauciinmune asociada a anticuerpos anticitoplasma de neutrófilos de patrón perinuclear (ANCA-P. El diagnóstico se estableció por resonancia nuclear magnética. Recibió tratamiento inmunosupresor con prednisona y ciclofosfamida con evolución favorable.Hypertrophic pachymeningitis is a very unusual disease, the main characteristic of which is thickening of the dura mater. We describe a patient who started this illness showing chronic headache and pauci-immune necrotizing extracapillary perinuclear antineutrophil cytoplasmic antibody (P-ANCA associated glomerulonephritis. The diagnosis was made by brain magnetic resonance image. She received immunosuppressant therapy with prednisonel and cyclophosphamide with clinical improvement.

  4. [Henoch-Schönlein purpura in a cocaine consumer man with HIV infection and ANCA-p positivity].

    Science.gov (United States)

    De Paoli, María C; Moretti, Dino; Scolari Pasinato, Carlos M; Buncuga, Martín G

    The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH.

  5. Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis

    Directory of Open Access Journals (Sweden)

    Nicholas D. Ward

    2014-01-01

    Full Text Available A rat model of antineutrophil cytoplasmic antibody (ANCA associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage and hemosiderosis with focal giant cells. In addition, small arteries revealed subtle neutrophil aggregation, and margination along vascular endothelium, but no definitive vasculitis. The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination. Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

  6. Evaluation of the FIDIS vasculitis multiplex immunoassay for diagnosis and follow-up of ANCA-associated vasculitis and Goodpasture's disease

    NARCIS (Netherlands)

    Damoiseaux, J.; Vaessen, M.; Knapen, Y.; Csernok, E.; Stegeman, C. A.; Van Paassen, P.; Tervaert, J. W. Cohen; Gershwin, ME; Shoenfeld, Y

    2007-01-01

    We have evaluated a new-multiplex immunoassay (FIDIS Vasculitis) for simultaneous detection and quantification of anti-MPO, -PR3, and -glomerular basement membrane (GBM) antibodies in diagnosis and follow-up of ANCA-associated vasculitides (AAV) and Goodpasture's disease. ANCA were determined in

  7. Comparison of a novel chemiluminescence enzyme immunoassay (CLEIA) with enzyme-linked immunosorbent assay (ELISA) for the determination of MPO-ANCA in patients with ANCA-associated vasculitis.

    Science.gov (United States)

    Hirose, Orie; Itabashi, Mitsuyo; Takei, Takashi; Nitta, Kosaku

    2015-03-01

    Myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA) represents the serological hallmark of ANCA-associated vasculitis (AAV). We evaluated the analytical and diagnostic accuracy of chemiluminescence enzyme immunoassay (CLEIA) versus enzyme-linked immunosorbent assay (ELISA) for the detection of MPO-ANCA. A total of 242 sera obtained from 51 patients with AAV and 103 patients without AAV were tested for MPO-ANCA by ELISA (NephroScholor MPOANC II) and CLEIA (the STACIA MEBLux test). Disease activity in the patients with AAV was determined based on the Birmingham Vasculitis Activity Score. We analyzed the correlations between the MPO-ANCA titers determined by the CLEIA and those determined by the ELISA, and also between the MPO-ANCA titers and the disease activity. The MPO-ANCA titers determined by the CLEIA (x) were strongly correlated with those determined by the ELISA (y). The correlation could be expressed by the following equation in this study: y = 1.8x + 7.7 (r = 0.96; p ELISA yielded positive test results in 57 of the 242 sera (23.6%). The CLEIA yielded false-positive test results in 4 of the 120 sera obtained from the non-AAV patients (3.3%), whereas the ELISA yielded a false-positive result in only 1 of the 120 sera obtained from the non-AAV patients (0.8%). The sensitivity and specificity of the CLEIA for the diagnosis of AAV were 100% and 96.7%, respectively, while those of the ELISA were 94.3% and 99.2%, respectively. The sensitivity and specificity of the CLEIA for the prediction of active disease were 100% and 64.4%, respectively, while those of the ELISA were 94.3% and 73.6%, respectively. The false positivity rate of the CLEIA for MPO-ANCA tended to be high as compared with that of the ELISA. Also, according to the correlation coefficient between the results of the CLEIA and the ELISA calculated in this study, it is necessary to pay attention to the differences in the sensitivity and specificity between CLEIA and ELISA.

  8. Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis

    Science.gov (United States)

    Monach, Paul A; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Cuthbertson, David; Krischer, Jeffrey; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Ikle, David; Kallenberg, Cees GM; Langford, Carol A; Mueller, Mark; Seo, Philip; St.Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Gu, Yi-Zhong; Snyder, Ronald D; Merkel, Peter A

    2011-01-01

    Objective To identify biomarkers that distinguish between active ANCA-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV. 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Serum levels of E-selectin, ICAM-3, MMP1, MMP3, MMP9, P-selectin, thrombomodulin, and VEGF were measured at study screening (time of active disease) and at month 6. ESR and CRP levels had been measured at the time of the clinical visit. The primary outcome was the difference in marker level between screening and month 6 among patients in remission (BVAS/WG score of 0) at month 6. Results All subjects had severe active vasculitis (mean BVAS/WG score 8.6 +/− 3.2 SD) at screening. Among the 123 subjects clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP3 levels were also higher among the 23 subjects with active disease at month 6 than among the 123 subjects in remission. MMP3 levels correlated weakly with ESR and CRP. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. PMID:21953143

  9. Outcome and Treatment of Elderly Patients with ANCA-Associated Vasculitis

    Science.gov (United States)

    Goh, Su Mein; Mohammad, Aladdin J.; Hruskova, Zdenka; Tanna, Anisha; Bruchfeld, Annette; Selga, Daina; Chocova, Zdenka; Westman, Kerstin; Eriksson, Per; Pusey, Charles D.; Tesar, Vladimir; Salama, Alan D.; Segelmark, Mårten

    2015-01-01

    Background and objectives ANCA-associated vasculitis is commonly found in elderly patients, but there are few data concerning outcome and treatment in the highest age groups. Design, setting, participants, & measurements Consecutive patients (N=151) presenting between 1997 and 2009 were retrospectively included from local registries in six centers in Sweden, the United Kingdom, and the Czech Republic if diagnosed with microscopic polyangiitis or granulomatosis with polyangiitis at age ≥75 years during the study period. Patients were followed until 2 years from diagnosis or death. Data on survival and renal function were analyzed with respect to age, sex, ANCA specificity, renal function, C-reactive protein, comorbidities, and Birmingham Vasculitis Activity Score at diagnosis as well as treatment during the first month. Results Median follow-up was 730 days (interquartile range, 244–730). Overall 1-year survival was 71.5% and 2-year survival was 64.6%. Older age, higher creatinine, and lower Birmingham Vasculitis Activity Score were associated with higher mortality in multivariable analysis. Patients who were not treated with standard immunosuppressive therapy had significantly worse survival. Renal survival was 74.8% at 1 year. No new cases of ESRD occurred during the second year. High creatinine at diagnosis was the only significant predictor of renal survival in multivariable analysis. Conclusions ANCA-associated vasculitis is a disease with substantial mortality and morbidity among elderly patients. This study showed a better prognosis for those who received immunosuppressive treatment and those who were diagnosed before having developed advanced renal insufficiency. PMID:26100457

  10. Hydralazine-associated adverse events: a report of two cases of hydralazine-induced ANCA vasculitis

    Directory of Open Access Journals (Sweden)

    Roman Zuckerman

    2018-05-01

    Full Text Available Abstract Hydralazine is a direct-acting vasodilator, which has been used in treatment for hypertension (HTN since the 1950s. While it is well known to cause drug-induced lupus (DIL, recent reports are indicating the emergence of the drug-induced anti-neutrophil cytoplasmic antibody (ANCA associated vasculitis (DIV. Herein, we describe two patients (aged 57 and 87 years who presented with severe acute kidney injury (AKI, proteinuria, and hematuria. Both were receiving hydralazine for the treatment of hypertension. ANCA serology was positive in both patients along with anti-histone antibodies (commonly seen in drug-induced vasculitis. Renal biopsy revealed classic crescentic (pauci-immune glomerulonephritis in these patients and hydralazine was discontinued. During the hospital course, the 57-year-old patient required dialysis therapy and was treated with steroids and rituximab for the ANCA disease. Renal function improved and the patient was discharged (off dialysis with a serum creatinine of 3.6 mg/dL (baseline = 0.9 mg/dL. At a follow-up of 2 years, the patient remained off dialysis with advanced chronic kidney disease (CKD (stage IIIb. The 87-year-old patient had severe AKI with serum creatinine at 10.41 mg/dL (baseline = 2.27 mg/dL. The patient required hemodialysis and was treated with steroids, rituximab, and plasmapheresis. Unfortunately, the patient developed catheter-induced bacteremia and subsequently died of sepsis. Hydralazine can cause severe AKI resulting in CKD or death. Given this extremely unfavorable adverse-event profile and the widespread availability of alternative anti-hypertensive agents, the use of hydralazine should be carefully considered.

  11. Long-term follow-up of cyclophosphamide compared with azathioprine for initial maintenance therapy in ANCA-associated vasculitis

    DEFF Research Database (Denmark)

    Walsh, M.; Faurschou, M.; Berden, A.

    2014-01-01

    BACKGROUND AND OBJECTIVES: Treatment with azathioprine within 3 months of remission induction with cyclophosphamide is a common treatment strategy for patients with ANCA-associated vasculitis. This study comprised patients undergoing long-term follow-up who were randomly allocated to azathioprine...... after 3-6 months or after 12 months of cyclophosphamide treatment. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Patients from 39 European centers between 1995 and 1997 with a new diagnosis of ANCA-associated vasculitis that involved the kidneys or another vital organ were eligible. At the time...

  12. Comparison of disease activity measures for anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis

    Science.gov (United States)

    Merkel, PA; Cuthbertson, DD; Hellmich, B; Hoffman, GS; Jayne, DRW; Kallenberg, CGM; Krischer, JP; Luqmani, R; Mahr, AD; Matteson, EL; Specks, U; Stone, JH

    2011-01-01

    Aim Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed. Methods A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment tools: the Birmingham Vasculitis Activity Score (BVAS), The BVAS for Wegener granulomatosis (BVAS/WG), BVAS 2003, a Physician Global Assessment (PGA), the Disease Extent Index (DEI) and the Five Factor Score (FFS). Five cases were rescored by all raters. Results Reliability of the measures was extremely high (intraclass correlations for the six measures all=0.98). Within each instrument, there were no significant differences or outliers among the scores from the 10 investigators. Test/retest reliability was high for each measure: range=0.77 to 0.95. The scores of the five acute activity measures correlated extremely well with one another. Conclusions Currently available tools for measuring disease extent and activity in ANCA-associated vasculitis are highly correlated and reliable. These results provide investigators with confidence to compare different clinical trial data and helps form common ground as international research groups develop new, improved and universally accepted vasculitis disease assessment instruments. PMID:18664546

  13. Cancer in ANCA-Associated Glomerulonephritis: A Registry-Based Cohort Study

    Directory of Open Access Journals (Sweden)

    Sanjeevan Sriskandarajah

    2017-01-01

    Full Text Available Background. Immunosuppressive therapy for antineutrophil cytoplasmic antibody-associated vasculitis has been associated with increased malignancy risk. Objectives. To quantify the cancer risk associated with contemporary cyclophosphamide-sparing protocols. Methods. Patients from the Norwegian Kidney Biopsy Registry between 1988 and 2012 who had biopsy-verified pauci-immune glomerulonephritis and positive antineutrophil cytoplasmic antibody (ANCA serology were included. Standardised incidence ratios (SIRs were calculated to compare the study cohort with the general population. Results. The study cohort included 419 patients. During 3010 person-years, cancer developed in 41 patients (9.79%; the expected number of cancer cases was 37.5 (8.95%. The cohort had SIRs as follows: 1.09, all cancer types (95% CI, 0.81 to 1.49; 0.96, all types except nonmelanoma skin cancer (95% CI, 0.69 to 1.34; 3.40, nonmelanoma skin cancer (95% CI, 1.62 to 7.14; 3.52, hematologic cancer (95% CI, 1.32 to 9.37; 2.12, posttransplant cancer (95% CI, 1.01 to 4.44; and 1.53, during the 1–5-year follow-up after diagnosis (95% CI, 1.01 to 2.32. Conclusions. Cancer risk did not increase significantly in this cohort with ANCA-associated glomerulonephritis. However, increased risk of nonmelanoma skin cancer, posttransplant cancer, and hematologic cancer indicates an association between immunosuppression and malignancy.

  14. Rituximab as maintenance therapy for ANCA associated vasculitis: how, when and why?

    Science.gov (United States)

    Alba, Marco A; Flores-Suárez, Luis Felipe

    2016-01-01

    ANCA-associated vasculitides (AAV) are chronic autoimmune diseases characterized by inflammation and destruction of small vessels. Rituximab is now licensed for use as a remission-induction agent in the treatment of these disorders. During recent years, several non-controlled studies have suggested that rituximab may be of value in maintaining disease remission in AAV. In these series, 3 techniques have been tried: "watch-and-wait", repeated cycles in fixed intervals, or administration based on proposed biomarkers. More importantly, the results of the MAINRITSAN trial showed that this anti-CD20 agent is superior to azathioprine for preventing major relapses in AAV. This review summarizes current information regarding the effectiveness, timing, dosing, duration and safety of rituximab as a valid option for remission maintenance. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  15. A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegener's Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies

    OpenAIRE

    Gmurczyk, Aleksandra; Ahya, Shubhada N.; Goldschmidt, Robert; Kim, George; Ho, L. Tammy; Nash, Kevin

    2010-01-01

    Wegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti–neutrophil cytoplasmic antibodies (ANCA), and approximately 10% have both circulating ANCA antibodies and concomitan...

  16. Clinical presentation and outcome prediction of clinical, serological, and histopathological classification schemes in ANCA-associated vasculitis with renal involvement.

    Science.gov (United States)

    Córdova-Sánchez, Bertha M; Mejía-Vilet, Juan M; Morales-Buenrostro, Luis E; Loyola-Rodríguez, Georgina; Uribe-Uribe, Norma O; Correa-Rotter, Ricardo

    2016-07-01

    Several classification schemes have been developed for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), with actual debate focusing on their clinical and prognostic performance. Sixty-two patients with renal biopsy-proven AAV from a single center in Mexico City diagnosed between 2004 and 2013 were analyzed and classified under clinical (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], renal limited vasculitis [RLV]), serological (proteinase 3 anti-neutrophil cytoplasmic antibodies [PR3-ANCA], myeloperoxidase anti-neutrophil cytoplasmic antibodies [MPO-ANCA], ANCA negative), and histopathological (focal, crescenteric, mixed-type, sclerosing) categories. Clinical presentation parameters were compared at baseline between classification groups, and the predictive value of different classification categories for disease and renal remission, relapse, renal, and patient survival was analyzed. Serological classification predicted relapse rate (PR3-ANCA hazard ratio for relapse 2.93, 1.20-7.17, p = 0.019). There were no differences in disease or renal remission, renal, or patient survival between clinical and serological categories. Histopathological classification predicted response to therapy, with a poorer renal remission rate for sclerosing group and those with less than 25 % normal glomeruli; in addition, it adequately delimited 24-month glomerular filtration rate (eGFR) evolution, but it did not predict renal nor patient survival. On multivariate models, renal replacement therapy (RRT) requirement (HR 8.07, CI 1.75-37.4, p = 0.008) and proteinuria (HR 1.49, CI 1.03-2.14, p = 0.034) at presentation predicted renal survival, while age (HR 1.10, CI 1.01-1.21, p = 0.041) and infective events during the induction phase (HR 4.72, 1.01-22.1, p = 0.049) negatively influenced patient survival. At present, ANCA-based serological classification may predict AAV relapses, but neither clinical nor serological

  17. Long-Term Maintenance Therapy Using Rituximab-Induced Continuous B-Cell Depletion in Patients with ANCA Vasculitis

    Science.gov (United States)

    Pendergraft, William F.; Cortazar, Frank B.; Wenger, Julia; Murphy, Andrew P.; Rhee, Eugene P.; Laliberte, Karen A.; Niles, John L.

    2014-01-01

    Background and objectives Remission in the majority of ANCA vasculitis patients is not sustained after a single course of rituximab, and risk of relapse warrants development of a successful strategy to ensure durable remission. Design, setting, participants, & measurements A retrospective analysis of ANCA vasculitis patients who underwent maintenance therapy using rituximab-induced continuous B-cell depletion for up to 7 years was performed. Maintenance therapy with rituximab was initiated after achieving remission or converting from other prior maintenance therapy. Continuous B-cell depletion was achieved in all patients by scheduled rituximab administration every 4 months. Disease activity, serologic parameters, adverse events, and survival were examined. Results In the study, 172 patients (mean age=60 years, 55% women, 57% myeloperoxidase–ANCA) treated from April of 2006 to March of 2013 underwent continuous B-cell depletion with rituximab. Median remission maintenance follow-up time was 2.1 years. Complete remission (Birmingham Vasculitis Activity Score [BVAS]=0) was achieved in all patients. Major relapse (BVAS≥3) occurred in 5% of patients and was associated with weaning of other immunosuppression drugs. Remission was reinduced in all patients. Survival mirrored survival of a general age-, sex-, and ethnicity-matched United States population. Conclusion This analysis provides evidence for long-term disease control using continuous B-cell depletion. This treatment strategy in ANCA vasculitis patients also seems to result in survival rates comparable with rates in a matched reference population. These findings suggest that prospective remission maintenance treatment trials using continuous B-cell depletion are warranted. PMID:24626432

  18. ANCA Associated Vasculitis and Renal Failure Related to Propylthiouracil and Hyperthyroidism Induced Cholestasis in the Same Case

    Directory of Open Access Journals (Sweden)

    Mehmet Tuncay

    2014-01-01

    Full Text Available Introduction. Liver involvement due to hyperthyroidism and also ANCA positive vasculitis related renal failure cases were reported separately several times before. However, to our knowledge, these two complications together in the same case had never been observed before. Case Presentation. The case of an ANCA positive 71-year-old Caucasian male with renal failure and lung involvement, subclinical hyperthyroidism, and intrahepatic cholestatic jaundice was presented in this paper. After exclusion of all of the other possibilities, cholestatic hepatitis was explained by subclinical hyperthyroidism; renal failure and lung involvement were interpreted as ANCA related vasculitis which might be a side effect of propylthiouracil use. Conclusion. The coexistence of these rare conditions in the same patient deserves emphasis and it is worth reporting. This case demonstrates that following the clinical course of the patient is essential after prescribing any medications to see whether any complication occurs or not. If the complications of this case were noticed earlier, it would be possible to treat and to prevent the permanent damages.

  19. MIHAELA PUŞCAŞ, Child Abuse. Forms, Motivation, Consequences

    Directory of Open Access Journals (Sweden)

    GHEORGHE FLORIAN

    2008-03-01

    Full Text Available Mass-media frequently bring before the public cases in which parents abuse their own children. Each time the reactions of citizens are intense and unanimous:anger, revolt, requests for extreme punishment, the right to complain against aggressive behavior occurring in their vicinity. Modern research on family revealed a world loaded with tensions and conflicts in which abuse is often presented disguised as love for children and the wish to provide a good education. This article approaches the psychological aspects of the various forms of child abuse, as these are treated in the specialized literature, trying to offer answers to legitimate questions: are these isolated cases or are we dealing with a real phenomenon; how spread is this phenomenon; do people today love their children less than before; is there a social environment which favors such behavior; how can abuse situations which are spread over years be explained; which are the visible signs that a child is being abused by his/her parents; what are the consequences on the development of personality; are there statistics for a larger period of time to help knowing whether the phenomenon is increasing or decreasing?

  20. Efficacy of Remission-Induction Regimens for ANCA-Associated Vasculitis

    Science.gov (United States)

    Specks, Ulrich; Merkel, Peter A.; Seo, Philip; Spiera, Robert; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St. Clair, E. William; Fessler, Barri J.; Ding, Linna; Viviano, Lisa; Tchao, Nadia K.; Phippard, Deborah J.; Asare, Adam L.; Lim, Noha; Ikle, David; Jepson, Brett; Brunetta, Paul; Allen, Nancy B.; Fervenza, Fernando C.; Geetha, Duvuru; Keogh, Karina; Kissin, Eugene Y.; Monach, Paul A.; Peikert, Tobias; Stegeman, Coen; Ytterberg, Steven R.; Mueller, Mark; Sejismundo, Lourdes P.; Mieras, Kathleen; Stone, John H.

    2018-01-01

    Background The 18-month efficacy of a single course of rituximab as compared with conventional immunosuppression with cyclophosphamide followed by azathioprine in patients with severe (organ-threatening) antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis is unknown. Methods In a multicenter, randomized, double-blind, double-dummy, noninferiority trial, we compared rituximab (375 mg per square meter of body-surface area administered once a week for 4 weeks) followed by placebo with cyclophosphamide administered for 3 to 6 months followed by azathioprine for 12 to 15 months. The primary outcome measure was complete remission of disease by 6 months, with the remission maintained through 18 months. Results A total of 197 patients were enrolled. As reported previously, 64% of the patients in the rituximab group, as compared with 53% of the patients in the cyclophosphamide–azathioprine group, had a complete remission by 6 months. At 12 and 18 months, 48% and 39%, respectively, of the patients in the rituximab group had maintained the complete remissions, as compared with 39% and 33%, respectively, in the comparison group. Rituximab met the prespecified criteria for noninferiority (P<0.001, with a noninferiority margin of 20%). There was no significant difference between the groups in any efficacy measure, including the duration of complete remission and the frequency or severity of relapses. Among the 101 patients who had relapsing disease at baseline, rituximab was superior to conventional immunosuppression at 6 months (P = 0.01) and at 12 months (P = 0.009) but not at 18 months (P = 0.06), at which time most patients in the rituximab group had reconstituted B cells. There was no significant between-group difference in adverse events. Conclusions In patients with severe ANCA-associated vasculitis, a single course of rituximab was as effective as continuous conventional immunosuppressive therapy for the induction and maintenance of remissions over the

  1. Randomized Trial of C5a Receptor Inhibitor Avacopan in ANCA-Associated Vasculitis.

    Science.gov (United States)

    Jayne, David R W; Bruchfeld, Annette N; Harper, Lorraine; Schaier, Matthias; Venning, Michael C; Hamilton, Patrick; Burst, Volker; Grundmann, Franziska; Jadoul, Michel; Szombati, István; Tesař, Vladimír; Segelmark, Mårten; Potarca, Antonia; Schall, Thomas J; Bekker, Pirow

    2017-09-01

    Alternative C activation is involved in the pathogenesis of ANCA-associated vasculitis. However, glucocorticoids used as treatment contribute to the morbidity and mortality of vasculitis. We determined whether avacopan (CCX168), an orally administered, selective C5a receptor inhibitor, could replace oral glucocorticoids without compromising efficacy. In this randomized, placebo-controlled trial, adults with newly diagnosed or relapsing vasculitis received placebo plus prednisone starting at 60 mg daily (control group), avacopan (30 mg, twice daily) plus reduced-dose prednisone (20 mg daily), or avacopan (30 mg, twice daily) without prednisone. All patients received cyclophosphamide or rituximab. The primary efficacy measure was the proportion of patients achieving a ≥50% reduction in Birmingham Vasculitis Activity Score by week 12 and no worsening in any body system. We enrolled 67 patients, 23 in the control and 22 in each of the avacopan groups. Clinical response at week 12 was achieved in 14 of 20 (70.0%) control patients, 19 of 22 (86.4%) patients in the avacopan plus reduced-dose prednisone group (difference from control 16.4%; two-sided 90% confidence limit, -4.3% to 37.1%; P =0.002 for noninferiority), and 17 of 21 (81.0%) patients in the avacopan without prednisone group (difference from control 11.0%; two-sided 90% confidence limit, -11.0% to 32.9%; P =0.01 for noninferiority). Adverse events occurred in 21 of 23 (91%) control patients, 19 of 22 (86%) patients in the avacopan plus reduced-dose prednisone group, and 21 of 22 (96%) patients in the avacopan without prednisone group. In conclusion, C5a receptor inhibition with avacopan was effective in replacing high-dose glucocorticoids in treating vasculitis. Copyright © 2017 by the American Society of Nephrology.

  2. Joint Damage in ANCA-Associated Systemic Vasculitis. Report II: Wegener’s and Churg-Strauss Granulomatous Polyangiitis

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    D.V. Pomazan

    2016-08-01

    Full Text Available Wegener’s (granulomatosis with polyangiitis, GPA and Churg-Strauss (eosinophilic polyangiitis, EPA vasculitis are treated as a single variant of systemic necrotizing granulomatosis vasculitis, associated with anti-neutrophil cytoplasmic antibodies (ANCA. There is an urgent need for further study of articular syndrome in patients with ANCA-SV. Objective: to evaluate the incidence and nature of the lesion of the joints at GPA and EPA, connection with extra-articular signs of the disease. Material and methods. The study involved 58 patients with ANCA-SV, among which there were 28 patients with GPA (16 men and 12 women aged from 17 to 70 years old and 30 with EPA (14 men and 16 women aged 19–70 years old.The average duration of the disease in the first and second groups was 4 years and 11 years, respectively. I, II and III degree activity of the GPA were in the ratio 1 : 6 : 7 and in the cases of EPA — 1 : 3 : 4. The lung pathology was diagnosed in all cases with EPA, in patients with GPA — in 68 % of cases. In addition, 2.3 times less frequently cutaneous syndrome was detected. Results. The lesion of the joints in the form of arthritis or arthralgia occurs in 1/2 of the number of patients with granulomatous ANCA-SV in the ratio of HPA to EPA as 1 : 2, which is associated with the severity of extra-articular manifestations of disease, and in cases of EPA — with the level of antibodies to proteinase-3. Patients with EPA significantly more frequently had lesions of the maxillary joints, digital joints of foot, ankle, metatarsophalangeal, hip, sacroiliac and vertebral, and the last 4 were not diagnosed in patients with HPA. Epiphyseal osteoporosis, subchondral sclerosis, osteocytes, artrocalcinosis and changes of the menisci horns of the knee-joints were observed only at EPA. Conclusions. The severity of arthropathy prevails in EPA, compared to GPA, that is associated with great inflammatory degenerative changes of the articular, but intra

  3. CanVasc recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides - Executive summary.

    Science.gov (United States)

    McGeoch, Lucy; Twilt, Marinka; Famorca, Leilani; Bakowsky, Volodko; Barra, Lillian; Benseler, Susan; Cabral, David A; Carette, Simon; Cox, Gerald P; Dhindsa, Navjot; Dipchand, Christine; Fifi-Mah, Aurore; Goulet, Michele; Khalidi, Nader; Khraishi, Majed M; Liang, Patrick; Milman, Nataliya; Pineau, Christian A; Reich, Heather; Samadi, Nooshin; Shojania, Kam; Taylor-Gjevre, Regina; Towheed, Tanveer E; Trudeau, Judith; Walsh, Michael; Yacyshyn, Elaine; Pagnoux, Christian

    2015-01-01

    The Canadian Vasculitis research network (CanVasc) is composed of physicians from different medical specialties, including rheumatology and nephrology and researchers with expertise in vasculitis. One of its aims was to develop recommendations for the diagnosis and management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides in Canada. This executive summary features the 19 recommendations and 17 statements addressing general AAV diagnosis and management, developed by CanVasc group based on a synthesis of existing international guidelines, other published supporting evidence and expert consensus considering the Canadian healthcare context.

  4. CanVasc Recommendations for the Management of Antineutrophil Cytoplasm Antibody (ANCA-Associated Vasculitides – Executive Summary

    Directory of Open Access Journals (Sweden)

    Lucy McGeoch

    2015-11-01

    Full Text Available The Canadian Vasculitis research network (CanVasc is composed of physicians from different medical specialties, including rheumatology and nephrology and researchers with expertise in vasculitis. One of its aims was to develop recommendations for the diagnosis and management of antineutrophil cytoplasm antibody (ANCA-associated vasculitides in Canada. This executive summary features the 19 recommendations and 17 statements addressing general AAV diagnosis and management, developed by CanVasc group based on a synthesis of existing international guidelines, other published supporting evidence and expert consensus considering the Canadian healthcare context.

  5. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis.

    Science.gov (United States)

    Foray, Nathalie; Hudali, Tamer; Papireddy, Muralidhar; Gao, John

    2016-01-01

    Background . Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation . A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Renal biopsy revealed crescentic glomerulonephritis (GN) pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV). Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion . Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

  6. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

    Directory of Open Access Journals (Sweden)

    Nathalie Foray

    2016-01-01

    Full Text Available Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical history consisted of newly diagnosed but untreated rheumatoid arthritis, hypertension, and non-insulin-dependent diabetes mellitus. He was found to have acute renal failure, proteinuria, and hypoglycemia. Standard therapy, including intravenous fluids, did not improve his acute renal failure. A vasculitis workup resulted in a positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA. Renal biopsy revealed crescentic glomerulonephritis (GN pauci-immune type, suggestive of MPO-ANCA-associated vasculitis (MPO-AAV. Treatment consisted of prednisone, cyclophosphamide, and seven cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he received hemodialysis for another week and continued treatment with cyclophosphamide and prednisone. Conclusion. Patients with longstanding rheumatoid arthritis may develop renal failure due to nonsteroidal anti-inflammatory medication use and AA type amyloidosis; however, necrotizing glomerulonephritis with crescent formation has been rarely reported. This stresses the importance of early recognition and swift initiation of treatment.

  7. ANCA-GBM dot-blot : Evaluation of an assay in the differential diagnosis of patients presenting with rapidly progressive glomerulonephritis

    NARCIS (Netherlands)

    Rutgers, Abraham; Damoiseaux, Jan; Roozendaal, Caroline; Limburg, Pieter C; Stegeman, Coen A; Tervaert, Jan Willem Cohen

    Rapidly progressive glomerulonephritis (RPGN) is characterized by rapid and progressive loss of renal function and the presence of crescentic glomerulonephritis (CGN). Early diagnosis and appropriate treatment is mandatory to prevent death and/or renal failure. We have evaluated an ANCA-GBM dot-blot

  8. Clinical presentation of Churg-Strauss syndrome in children. : A 12-year-old-boy with ANCA-negative Churg-Strauss syndrome.

    NARCIS (Netherlands)

    F.G.E.M. Razenberg (Femke); J.W.C.M. Heynens (Jan); G. Jan de Vries (Geeuwke); L. Duijts (Liesbeth); J.C. de Jongste (Johan); J. de Blic (Jacques); P.P.R. Rosias (Philippe)

    2012-01-01

    textabstractChurg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The

  9. Androgen deficiency in male patients diagnosed with ANCA-associated vasculitis : A cause of fatigue and reduced health-related quality of life?

    NARCIS (Netherlands)

    Tuin, Janneke; Sanders, Jan-Stephan F.; Buhl, Birgit M.; van Beek, Andre P.; Stegeman, Coen A.

    2013-01-01

    Introduction: Low testosterone levels in men are associated with fatigue, limited physical performance and reduced health-related quality of life (HRQOL); however, this relationship has never been assessed in patients with anti-neutrophil cytoplasmic antibodies (ANCA) -associated vasculitides (AAV).

  10. Relato de Caso - Nefropatia de IgA associada ao ANCA com evolução favorável

    Directory of Open Access Journals (Sweden)

    Flávia Lara Barcelos

    2015-09-01

    Full Text Available ResumoIntrodução:Os anticorpos anticitoplasma de neutrófilos (ANCA comumente estão relacionados a glomerulonefrites rapidamente progressivas (GnRP com padrão pauci-imune. Apesar disso, a literatura mostra uma incidência além da esperada de ANCA nas GnRP por imunocomplexos. A nefropatia por imunoglobulina A (NIgA crescêntica é uma das GnRP que pode se associar ao ANCA.Objetivo:Relatar caso de NigA com sinais clínicos de mau prognóstico associado ao ANCA com evolução favorável após imunossupressão.Método:Foi relatado caso de paciente com 38 anos com quadro de hipertensão arterial (HAS, insuficiência renal (CKD-EPI- 37 ml/min/1,73 m2, proteinúria subnefrótica e hematúria. Nos antecedentes pessoais, relatava epistaxes ocasionais, rinossinusite e episódio de artrite com remissão espontânea. Durante a investigação diagnóstica, foram detectados ANCA positivo 1/160 e anti-PR3, porém, com biópsia renal compatível com NIgA com 38% de crescentes na amostra. Foi realizado diagnóstico de NIgA associada ao ANCA, sendo indicado tratamento imunossupressor por seis meses com corticoterapia (pulsoterapia com metilprednisolona 1 g por 3 dias, seguido de prednisona 1 mg/kg/dia e ciclofosfamida (500 mg com aumento crescente da dose até 750 mg/m2. Paciente evolui com recuperação da função renal, além da redução da proteinúria e da titulação de ANCA.Conclusão:A importância da identificação dessa sobreposição está no comportamento agressivo dessa doença caracterizada pela presença de crescentes, atrofia tubular e disfunção renal que podem regredir com início precoce da imunossupressão.

  11. Serum proteins reflecting inflammation, injury and repair as biomarkers of disease activity in ANCA-associated vasculitis

    Science.gov (United States)

    Monach, Paul A; Warner, Roscoe L; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Iklé, David; Kallenberg, Cees GM; Krischer, Jeffrey; Langford, Carol A; Mueller, Mark; Seo, Philip; St. Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Johnson, Kent J; Merkel, Peter A

    2016-01-01

    Objective To identify circulating proteins that distinguish between active anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner complementary to markers of systemic inflammation. Methods Twenty-eight serum proteins representing diverse aspects of the biology of AAV were measured before and 6 months after treatment in a large clinical trial of AAV. Subjects (n=186) enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were available for comparison. The primary outcome was the ability of markers to distinguish severe AAV (Birmingham Vasculitis Activity Score for Wegener’s granulomatosis (BVAS/WG)≥3 at screening) from remission (BVAS/WG=0 at month 6), using areas under receiver operating characteristic (ROC) curve (AUC). Results All subjects had severe active vasculitis (median BVAS/WG=8) at screening. In the 137 subjects in remission at month 6, 24 of the 28 markers showed significant declines. ROC analysis indicated that levels of CXCL13 (BCA-1), matrix metalloproteinase-3 (MMP-3) and tissue inhibitor of metalloproteinases-1 (TIMP-1) best discriminated active AAV from remission (AUC>0.8) and from healthy controls (AUC>0.9). Correlations among these markers and with ESR or CRP were low. Conclusions Many markers are elevated in severe active AAV and decline with treatment, but CXCL13, MMP-3 and TIMP-1 distinguish active AAV from remission better than the other markers studied, including ESR and CRP. These proteins are particularly promising candidates for future studies to address unmet needs in the assessment of patients with AAV. PMID:22975753

  12. Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.

    Science.gov (United States)

    McAdoo, Stephen P; Tanna, Anisha; Hrušková, Zdenka; Holm, Lisa; Weiner, Maria; Arulkumaran, Nishkantha; Kang, Amy; Satrapová, Veronika; Levy, Jeremy; Ohlsson, Sophie; Tesar, Vladimir; Segelmark, Mårten; Pusey, Charles D

    2017-09-01

    Co-presentation with both ANCA and anti-GBM antibodies is thought to be relatively rare. Current studies of such 'double-positive' cases report small numbers and variable outcomes. To study this further we retrospectively analyzed clinical features and long-term outcomes of a large cohort of 568 contemporary patients with ANCA-associated vasculitis, 41 patients with anti-GBM disease, and 37 double-positive patients with ANCA and anti-GBM disease from four European centers. Double-positive patients shared characteristics of ANCA-associated vasculitis (AAV), such as older age distribution and longer symptom duration before diagnosis, and features of anti-GBM disease, such as severe renal disease and high frequency of lung hemorrhage at presentation. Despite having more evidence of chronic injury on renal biopsy compared to patients with anti-GBM disease, double-positive patients had a greater tendency to recover from being dialysis-dependent after treatment and had intermediate long-term renal survival compared to the single-positive patients. However, overall patient survival was similar in all three groups. Predictors of poor patient survival included advanced age, severe renal failure, and lung hemorrhage at presentation. No single-positive anti-GBM patients experienced disease relapse, whereas approximately half of surviving patients with AAV and double-positive patients had recurrent disease during a median follow-up of 4.8 years. Thus, double-positive patients have a truly hybrid disease phenotype, requiring aggressive early treatment for anti-GBM disease, and careful long-term follow-up and consideration for maintenance immunosuppression for AAV. Since double-positivity appears common, further work is required to define the underlying mechanisms of this association and define optimum treatment strategies. Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  13. Androgen deficiency in male patients diagnosed with ANCA-associated vasculitis: a cause of fatigue and reduced health-related quality of life?

    OpenAIRE

    Tuin, Janneke; Sanders, Jan-Stephan F; Buhl, Birgit M; van Beek, André P; Stegeman, Coen A

    2013-01-01

    Introduction: Low testosterone levels in men are associated with fatigue, limited physical performance and reduced health-related quality of life (HRQOL); however, this relationship has never been assessed in patients with anti-neutrophil cytoplasmic antibodies (ANCA) -associated vasculitides (AAV). The aim of this study was to assess the prevalence of androgen deficiency and to investigate the role of testosterone in fatigue, limited physical condition and reduced HRQOL in men with AAV. Meth...

  14. Employment, work disability and quality of life in patients with ANCA-associated vasculitides. The EXPOVAS study.

    Science.gov (United States)

    Benarous, Lucas; Terrier, Benjamin; Laborde-Casterot, Hervé; Bérezné, Alice; Dunogué, Bertrand; Cohen, Pascal; Puéchal, Xavier; Mouthon, Luc; Bensefa-Colas, Lynda; Guillevin, Loic

    2017-01-01

    Improved therapeutic strategies for ANCA-associated vasculitis (AAV) have transformed acute and life-threatening diseases into chronic ones responsible for marked morbidity that could impact employment, work disability and quality of life (QoL). We aimed to analyse work, handicaps and QoL of AAV patients and identify their determinants. Patients with AAV were included in a cross-sectional study assessing employment, work disability and QoL. Specific and non-specific questionnaires, including SF-36, were sent to patients, and clinical-biological data that could affect QoL and their determinants were analysed. Questionnaires were completed by 189 patients. Among 94 working-age (employed; 23% of workers felt that their disease qualitatively limited the nature of their work, while 43% felt it limited the quantity of work they could do; 50% thought their disease had hindered their careers and 43% that it had led to a salary reduction. These results were comparable for the different vasculitides. QoL was significantly impaired for AAV patients compared to the general population (pemployment seemed to be preserved for the majority of the patients.

  15. T1 and T2 mapping for evaluation of myocardial involvement in patients with ANCA-associated vasculitides.

    Science.gov (United States)

    Greulich, Simon; Mayr, Agnes; Kitterer, Daniel; Latus, Joerg; Henes, Joerg; Steubing, Hannah; Kaesemann, Philipp; Patrascu, Alexandru; Greiser, Andreas; Groeninger, Stefan; Braun, Niko; Alscher, M Dominik; Sechtem, Udo; Mahrholdt, Heiko

    2017-01-06

    Myocardial involvement in AAV patients might be silent, presenting with no or nonspecific symptoms, normal ECG, and preserved left-ventricular ejection fraction (LV-EF). Since up to 50% of deaths in these patients may be due to myocardial involvement, a reliable diagnostic tool is warranted. In contrast to LGE-CMR, which has its strengths in detecting focal inflammatory or fibrotic processes, recent mapping techniques are able to detect even subtle, diffuse inflammatory or fibrotic processes. Our study sought to investigate ANCA (antineutrophil cytoplasmic antibody) associated vasculitides (AAV) patients for myocardial involvement by a cardiovascular magnetic resonance (CMR) protocol, including late gadolinium enhancement (LGE) and mapping sequences. Thirty seven AAV patients were prospectively enrolled and underwent CMR imaging. Twenty healthy volunteers served as controls. Mean LV-EF was 64%; LGE prevalence of the AAV patients was 43%. AAV patients had higher median native T1 (988 vs. 952 ms, p T2 (53 vs. 49 ms, p T2 in AAV patients showed the highest prevalence of abnormally increased values beyond the 95% percentile of controls. AAV patients demonstrated increased T1, ECV, and T2 values, with native T1 and T2 showing the highest prevalence of values beyond the 95% percentile of normal. Since these findings seem to be independent of LGE, mapping techniques may provide complementary information to LGE-CMR in the assessment of myocardial involvement in patients with AAV.

  16. Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin.

    Science.gov (United States)

    Richter, C; Schnabel, A; Csernok, E; De Groot, K; Reinhold-Keller, E; Gross, W L

    1995-07-01

    In this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30 g/day over 5 days. Clinical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.

  17. The 12th Edition of the Scientific Days of the National Institute for Infectious Diseases “Prof. Dr. Matei Bals” and the 12th National Infectious Diseases Conference

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    Cristian-Mihail Niculae

    2016-11-01

    âlțea, Livia Trașcă, Monica Cîrstoiu A91 Acute respiratory failure in critical patients with sepsis Doina Viorica Iovănescu, Cleo Nicoleta Roșculeț, Andrei Rogoz, Cătălin Gabriel Apostolescu, Viorica Daniela Mitescu, Tudor Gheorghe Vladoiu, Dalila Toma, Catrinel Ciuca A92 Cochleo-vestibular deficit secondary to Granulicatella elegans meningitis Mădălina Georgescu A93 Influenza 2015/2016 – clinical, epidemiological and virological characteristics of cases admitted in three infectious diseases hospitals Daniela Pițigoi, Alina Elena Ivanciuc, Mihaela Lazar, Teodora Ionescu, Carmen Maria Cherciu, Cristina Țecu, Maria Elena Mihai, Maria Nițescu, Rodica Bacruban, Delia Azamfire, Aura Dumitrescu, Elena Ianosik, Daniela Leca, Elena Duca, Andra Teodor, Codrina Bejan, Emanoil Ceaușu, Simin-Aysel Florescu, Corneliu Popescu, Grațiela Târdei, Codrina Juganariu, Emilia Lupulescu A94 Severe complications of varicella requiring hospitalization in previously healthy children in Brașov county Ligia Rodina, Maria Elena Cocuz A95 Clinical forms of Clostridium difficile colitis in children Gheorghiță Jugulete, Adina Stăncescu, Cristina Elena Popescu, Luminița Marin, Diana Zaharia, Cristina Dumitrescu, Endis Osman A96 Community-acquired pneumonia – demographic, clinical and etiological aspects Irina Niculescu, Augustin Cupșa, Iulian Diaconescu, Florentina Dumitrescu, Livia Dragonu, Andreea Stoian, Lucian Giubelan, Cristina Roskanovic A97 Acute myocarditis in an adult patient with chickenpox - Case report Ramona-Alexandra Zamfir, Mihaela Ionica, Otilia-Elisabeta Benea A98 Caustic oropharyngeal wound with acute group F streptococcal superinfection mimicking diphtheria – case report and differential diagnosis Maria-Cristina Sîrbu, AnaMaria Dobrotă, Alina Cristina Neguț, Roxana Duda, Rodica Bacruban, Daniela Pițigoi, Cristiana Cerasella Dragomirescu, Daniela Tălăpan, Olga Dorobăț, Adrian Streinu-Cercel, Anca Streinu-Cercel A99 Clostridium difficile infection in HIV

  18. Clinical presentation of Churg-Strauss syndrome in children: A 12-year-old-boy with ANCA-negative Churg-Strauss syndrome.

    Science.gov (United States)

    Razenberg, Femke G E M; Heynens, Jan W C M; Jan de Vries, Geeuwke; Duijts, Liesbeth; de Jongste, Johan C; de Blic, Jacques; Rosias, Philippe P R

    2012-01-01

    Churg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The propositus included, 50 cases of childhood Churg-Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg-Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome.

  19. A case of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA positive/IgG4-related lung disease

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    Hirokazu Touge

    2017-01-01

    Full Text Available IgG4-related lung disease (IgG4-RLD is a rare and chronic progressive autoimmune disease. We report a case of IgG4-related inflammatory pseudo-tumor of the lung that was seropositive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA. A 61-year-old male had a mass lesion in the right lower lung field in chest X-ray. Transbronchial lung biopsy resulted in a pathological diagnosis of IgG4-RLD. The condition was improved by hormonal therapy.

  20. CERTIFICATION REPORT The certification of the mass concentration of immunoglobulin G proteinase 3 anti-neutrophil cytoplasmic autoantibodies (IgG PR3 ANCA) in human serum: ERM® - DA483/IFCC

    OpenAIRE

    MONOGIOUDI EVANTHIA; HUTU DANA PETRONELA; CHAROUD-GOT JEAN; SHELDON JOANNA; SCHIMMEL HEINZ; TRAPMANN STEFANIE; MERONI PIERLUIGI; EMONS HENDRIK; ZEGERS INGRID

    2017-01-01

    This report describes the production and certification of ERM-DA483/IFCC, a serum protein reference material intended for the standardisation of measurements of immunoglobulin G proteinase 3 anti-neutrophil cytoplasmic autoantibodies (IgG PR3 ANCA). The material was produced according to ISO Guide 34:2009 [ ] and is certified in accordance with ISO Guide 35:2006. The raw material used to prepare ERM-DA483/IFCC was a plasmapheresis material containing a high concentration of IgG PR3 ANCA. A...

  1. Anti-hLAMP2-antibodies and dual positivity for anti-GBM and MPO-ANCA in a patient with relapsing pulmonary-renal syndrome

    Directory of Open Access Journals (Sweden)

    Kistler Thomas

    2011-06-01

    Full Text Available Abstract Background Pulmonary-renal syndrome associated with anti-glomerular basement membrane (GBM antibodies, also known as Goodpasture's syndrome, is a rare but acute and life-threatening condition. One third of patients presenting as anti-GBM antibody positive pulmonary-renal syndrome or rapidly progressive glomerulonephritis are also tested positive for anti-neutrophil cytoplasmic antibodies (ANCA. Whilst anti-GBM disease is considered a non-relapsing condition, the long-term course of double-positive patients is less predictable. Case Presentation We report a patient with such dual positivity, who presented with pulmonary hemorrhage, crescentic glomerulonephritis and membranous nephropathy. Plasmapheresis in combination with immunosuppresive therapy led to a rapid remission but the disease relapsed after two years. The serum of the patient was tested positive for antibodies to human lysosomal membrane protein 2 (hLAMP2, a novel autoantigen in patients with active small-vessel vasculitis (SVV. The anti-hLAMP2 antibody levels correlated positively with clinical disease activity in this patient. Conclusion We hypothesize that this antibody may indicate a clinical course similar to ANCA-associated vasculitis in double-positive patients. However, this needs to be confirmed on comprehensive patient cohorts.

  2. Low Serum Complement C3 Levels at Diagnosis of Renal ANCA-Associated Vasculitis Is Associated with Poor Prognosis.

    Directory of Open Access Journals (Sweden)

    Jean-François Augusto

    Full Text Available Recent studies have demonstrated the key role of the complement alternative pathway (cAP in the pathophysiology of experimental ANCA-associated vasculitis (AAV. However, in human AAV the role of cAP has not been extensively explored. In the present work, we analysed circulating serum C3 levels measured at AAV onset and their relation to outcomes.We conducted a retrospective observational cohort study including 45 consecutive patients with AAV diagnosed between 2000 and 2014 with serum C3 measurement at diagnosis, before immunosuppressive treatment initiation. Two groups were defined according to the median serum C3 level value: the low C3 group (C3<120 mg/dL and the high C3 level group (C3≥120 mg/dL. Patient and renal survivals, association between C3 level and renal pathology were analysed.Serum complement C3 concentration remained in the normal range [78-184 mg/dL]. Compared with the high C3 level, the patients in the low C3 level group had lower complement C4 concentrations (P = 0.008 and lower eGFR (P = 0.002 at diagnosis. The low C3 level group had poorer patient and death-censored renal survivals, compared with the high C3 level group (P = 0.047 and P = 0.001, respectively. We observed a significant negative correlation between C3 levels and the percentage of glomeruli affected by cellular crescent (P = 0.017, r = -0.407. According to the Berden et al renal histologic classification, patients in the crescentic/mixed category had low C3 levels more frequently (P<0.01. Interestingly, we observed that when patients with the crescentic/mixed histologic form were analysed according to C3 level, long term renal survival was significantly greater in the high C3 level group than in the low C3 level group (100% vs 40.7% at 6 years, p = 0.046. No relationship between serum C4 and renal outcome was observed.A Low C3 serum level in AAV patients at diagnosis is associated with worse long-term patient and renal survival.

  3. Development and Validation of Case-Finding Algorithms for the Identification of Patients with ANCA-Associated Vasculitis in Large Healthcare Administrative Databases

    Science.gov (United States)

    Sreih, Antoine G.; Annapureddy, Narender; Springer, Jason; Casey, George; Byram, Kevin; Cruz, Andy; Estephan, Maya; Frangiosa, Vince; George, Michael D.; Liu, Mei; Parker, Adam; Sangani, Sapna; Sharim, Rebecca; Merkel, Peter A.

    2016-01-01

    Purpose To develop and validate case-finding algorithms for granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA). Methods 250 patients per disease were randomly selected from 2 large healthcare systems using the International Classification of Diseases version 9 (ICD9) codes for GPA/EGPA (446.4) and MPA (446.0). 16 case-finding algorithms were constructed using a combination of ICD9 code, encounter type (inpatient or outpatient), physician specialty, use of immunosuppressive medications, and the anti-neutrophil cytoplasmic antibody (ANCA) type. Algorithms with the highest average positive predictive value (PPV) were validated in a third healthcare system. Results An algorithm excluding patients with eosinophilia or asthma and including the encounter type and physician specialty had the highest PPV for GPA (92.4%). An algorithm including patients with eosinophilia and asthma and the physician specialty had the highest PPV for EGPA (100%). An algorithm including patients with one of the following diagnoses: alveolar hemorrhage, interstitial lung disease, glomerulonephritis, acute or chronic kidney disease, the encounter type, physician specialty, and immunosuppressive medications had the highest PPV for MPA (76.2%). When validated in a third healthcare system, these algorithms had high PPV (85.9% for GPA, 85.7% for EGPA, and 61.5% for MPA). Adding the ANCA type increased the PPV to 94.4%, 100%, and 81.2% for GPA, EGPA, and MPA respectively. Conclusion Case-finding algorithms accurately identify patients with GPA, EGPA, and MPA in administrative databases. These algorithms can be used to assemble population-based cohorts and facilitate future research in epidemiology, drug safety, and comparative effectiveness. PMID:27804171

  4. A Candidate Gene Approach to ANCA-Associated Vasculitis Reveals Links to the C3 and CTLA-4 Genes but not to the IL1-Ra And Fcγ-RIIa Genes.

    OpenAIRE

    Persson, Ulf; Gullstrand, Birgitta; Pettersson, Åsa; Sturfelt, Gunnar; Truedsson, Lennart; Segelmark, Mårten

    2013-01-01

    Background/Aims: The aim of the study is to search for associations between Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and polymorphisms in the genes of four key molecules possibly involved in different pathogenic pathways; complement C3, CTLA-4, Fcγ-RIIa and IL1-Ra. Patients and Methods: Patients with AAV (n=105) subgrouped as microscopic polyangiitis or granulomatosis with polyangiitis (Wegener's granulomatosis) and myeloperoxidase (MPO) or proteinase 3 (PR3) A...

  5. Leukocyte and serum S100A8/S100A9 expression reflects disease activity in ANCA-associated vasculitis and glomerulonephritis

    Science.gov (United States)

    Pepper, Ruth J; Hamour, Sally; Chavele, Konstantia-Maria; Todd, Sarah K; Rasmussen, Niels; Flint, Shaun; Lyons, Paul A; Smith, Kenneth G C; Pusey, Charles D; Cook, H Terence; Salama, Alan D

    2013-01-01

    Antineutrophil cytoplasm antibody (ANCA)–associated vasculitis (AAV) commonly results in glomerulonephritis, in which neutrophils and monocytes have important roles. The heterodimer calprotectin (S100A8/S100A9, mrp8/14) is a Toll-like receptor-4 ligand found in neutrophils and monocytes and is elevated in inflammatory conditions. By immunohistochemistry of renal biopsies, patients with focal or crescentic glomerular lesions were found to have the highest expression of calprotectin and those with sclerotic the least. Serum levels of calprotectin as measured by ELISA were elevated in patients with active AAV and the levels decreased but did not normalize during remission, suggesting subclinical inflammation. Calprotectin levels in patients with limited systemic disease increased following treatment withdrawal and were significantly elevated in patients who relapsed compared with those who did not. As assessed by flow cytometry, patients with AAV had higher monocyte and neutrophil cell surface calprotectin expression than healthy controls, but this was not associated with augmented mRNA expression in CD14+ monocytes or CD16+ neutrophils. Thus, serum calprotectin is a potential disease biomarker in patients with AAV, and may have a role in disease pathogenesis. PMID:23423260

  6. CD4+CD28null T Cells are related to previous cytomegalovirus infection but not to accelerated atherosclerosis in ANCA-associated vasculitis.

    Science.gov (United States)

    Slot, Marjan C; Kroon, Abraham A; Damoiseaux, Jan G M C; Theunissen, Ruud; Houben, Alfons J H M; de Leeuw, Peter W; Tervaert, Jan Willem Cohen

    2017-05-01

    Previous studies have suggested an increased risk for cardiovascular events in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). We analyzed the presence of atherosclerotic damage in patients with AAV in relation to the presence of CD4 + CD28 null T cells and antibodies against cytomegalovirus (CMV) and human Heat-Shock Protein 60 (hHSP60). In this cross-sectional study, patients with inactive AAV were compared with healthy controls (HC). Carotid intima-media thickness (IMT) and aortic pulse-wave velocity (PWV) were measured. In addition, CD4 + CD28 null T cells, anti-CMV, and anti-hHSP60 levels were determined. Forty patients with AAV were included. Patients' spouses were recruited as HC (N = 38). CD4 + CD28 null T cells are present in patients with AAV in a higher percentage (median 3.1, range 0.01-85) than in HC (0.28, 0-36, P CD4 + CD28 null T cells (0.33 vs 13.8, P CD4 + CD28 null T cells and/or a previous CMV infection and IMT or PWV. There was no relation between anti-hHSP60 and CD4 + CD28 null T cells. Increased PWV values suggest atherosclerotic damage in patients with AAV. Plaque size, as determined by IMT, did not differ. CD4 + CD28 null T cells are increased in AAV and related to the previous CMV infection.

  7. Androgen deficiency in male patients diagnosed with ANCA-associated vasculitis: a cause of fatigue and reduced health-related quality of life?

    Science.gov (United States)

    Tuin, Janneke; Sanders, Jan-Stephan F; Buhl, Birgit M; van Beek, André P; Stegeman, Coen A

    2013-01-01

    Low testosterone levels in men are associated with fatigue, limited physical performance and reduced health-related quality of life (HRQOL); however, this relationship has never been assessed in patients with anti-neutrophil cytoplasmic antibodies (ANCA) -associated vasculitides (AAV). The aim of this study was to assess the prevalence of androgen deficiency and to investigate the role of testosterone in fatigue, limited physical condition and reduced HRQOL in men with AAV. Male patients with AAV in remission were included in this study. Fatigue and HRQOL were assessed by the multi-dimensional fatigue inventory (MFI)-20 and RAND-36 questionnaires. Seventy male patients with a mean age of 59 years (SD 12) were included. Scores of almost all subscales of both questionnaires were significantly worse in patients compared to controls. Mean total testosterone and free testosterone levels were 13.8 nmol/L (SD 5.6) and 256 pmol/L (SD 102), respectively. Androgen deficiency (defined according to Endocrine Society Clinical Practice Guidelines) was present in 47% of patients. Scores in the subscales of general health perception, physical functioning and reduced activity were significantly worse in patients with androgen deficiency compared to patients with normal androgen levels. Testosterone and age were predictors for the RAND-36 physical component summary in multiple linear regression analysis. Testosterone, age, vasculitis damage index (VDI) and C-reactive protein (CRP) were associated with the MFI-20 subscale of general fatigue. This study showed that androgen deficiency was present in a substantial number of patients with AAV. Testosterone was one of the predictors for physical functioning and fatigue. Testosterone may play a role in fatigue, reduced physical performance and HRQOL in male patients with AAV.

  8. Procena uticaja udesa sa opasnim materijama na kasarnu "Car Lazar" u Kruševcu / Assessment of the impact of accidents involving hazardous substances on Car Lazar military barracks in Kruševac / Прогноз воздействия аварии транспорта с опасным грузом на казарму «Цар Лазар» в городе Крушевац

    Directory of Open Access Journals (Sweden)

    Srđan Z. Rutić

    2016-01-01

    Full Text Available U radu je izvršena analiza rizika od udesa izazvanih opasnim materijama u drumskom i železničkom saobraćaju u gradu Kruševcu. Definisane su kritične tačke i izvršena je analiza mogućnosti nastanka udesa pri transportu opasnih materija, kao i procena njihovog uticaja na kasarnu „Car Lazar” u Kruševcu. Zaključeno je da je kasarna veoma ugrožena od mogućnosti nastanka udesa izazvanih opasnim materijama u drumskom i železničkom saobraćaju, pa naročitu pažnju treba obratiti na obezbeđenje od takvih udesa. / The paper analyzes the risk of accidents involving hazardous substances during road and rail traffic in the city of Kruševac. Critical points in road and rail transport in the city of Kruševac are defined, followed by an analysis of the possibility of accidents in the transport of dangerous goods as well as an assessment of their impact on Car Lazar military barracks in Kruševac. It was concluded that Car Lazar barracks in Kruševac are significantly threatened by a possibility of accidents with hazardous materials in road and rail transport; therefore, particular attention should be paid to the prevention from accidents involving hazardous substances. / В данной работе выполнен прогноз рисков от железнодорожной и автодорожной аварий транспорта с опасным грузом на территории города Крушевац. Определены критические пункты железнодорожного и автодорожного маршрута в городе Крушевац, выполнен анализ рисков от аварий железнодорожного и автодорожного транспорта с опасным грузом, а также выполнен прогноз последствий аварии для казармы «Цар Лазар» в городе Крушевац.

  9. ANCA-associated vasculitis and malignancy

    DEFF Research Database (Denmark)

    Mahr, Alfred; Heijl, Caroline; Le Guenno, Guillaume

    2013-01-01

    of individual therapeutic agents is difficult to dissect, but cyclophosphamide has emerged as a major contributor to cancer development because of its direct carcinogenic properties. Awareness of cancer risk in AAV calls for increased implementation of measures to prevent or screen for cancer and development......In this review, we summarise the current understanding of the potential link between cancer and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (Wegener's; GPA) and microscopic polyangiitis (MPA). As is true for many autoimmune...... or inflammatory rheumatic diseases, AAV diagnosis and therapy are associated with an increased risk of de novo cancer development, likely as a result of impaired immunosurveillance, direct oncogenicity of immunosuppressive agents and perhaps malignant degeneration of tissues undergoing chronic immune stimulation...

  10. Mõiste saddiq/dikaios arengust : katse rakendada Lazar Gulkowitschi mõisteloolist meetodit algkristluse uurimisel / Urmas Nõmmik

    Index Scriptorium Estoniae

    Nõmmik, Urmas, 1975-

    2009-01-01

    Mõiste "õige inimene" kasutusest Vanas ja Uues Testamendis, apokrüüfses kirjanduses ning Qumrani kogudes. Septuaginta (ladina keeles 'seitsekümmend') on heebreakeelse Vana Testamendi vanim teadaolev täielik tõlge kreeka keelde

  11. Makedoonia veetis tänase öö kreeklastega tüli lahendades / Lazar Elenovski ; interv. Evelyn Kaldoja

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    Elenovski, Lazar

    2008-01-01

    Makedoonia kaitseminister räägib Bukaresti tippkohtumisel oma riigi pürgimisest NATO-sse, tingimuste täitmisest ja läbirääkimistest ning Kreeka vastuseisust NATO-sse pääsemisel nende maakonnaga sarnase nime tõttu. Vt. samas: Berliin ja Pariis on MAPide suhtes resoluutsed

  12. Contribution by Lazare and Sadi Carnot to the caloric theory of heat and its inspirative role in thermodynamics

    Czech Academy of Sciences Publication Activity Database

    Šesták, Jaroslav; Mareš, Jiří J.; Hubík, Pavel; Proks, I.

    2009-01-01

    Roč. 97, č. 2 (2009), s. 679-683 ISSN 1388-6150 R&D Projects: GA AV ČR IAA1010404; GA AV ČR IAA100100639 Institutional research plan: CEZ:AV0Z10100521 Keywords : caloric as entropy * Carnot * efficiency * history * thermal analysis * thermodynamics Subject RIV: BM - Solid Matter Physics ; Magnetism Impact factor: 1.587, year: 2009

  13. Using Mass Spectrometry to Quantify Rituximab and Perform Individualized Immunoglobulin Phenotyping in ANCA-Associated Vasculitis

    NARCIS (Netherlands)

    Mills, John R.; Cornec, Divi; Dasari, Surendra; Ladwig, Paula M.; Hummel, Amber M.; Cheu, Melissa; Murray, David L.; Willrich, Maria A.; Snyder, Melissa R.; Hoffman, Gary S.; Kallenberg, Cees G. M.; Langford, Carol A.; Merkel, Peter A.; Monach, Paul A.; Seo, Philip; Spiera, Robert F.; St Cair, E. William; Stone, John H.; Specks, Ulrich; Barnidge, David R.

    2016-01-01

    Therapeutic monoclonal immunoglobulins (mAbs) are used to treat patients with a wide range of disorders including autoimmune diseases. As pharmaceutical companies bring more fully humanized therapeutic mAb drugs to the healthcare market analytical platforms that perform therapeutic drug monitoring

  14. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts

    Science.gov (United States)

    Pagnoux, Christian; Carette, Simon; Khalidi, Nader A.; Walsh, Michael; Hiemstra, Thomas F.; Cuthbertson, David; Langford, Carol; Hoffman, Gary S.; Koening, Curry L.; Monach, Paul A.; Moreland, Larry; Mouthon, Luc; Seo, Phil; Specks, Ulrich; Ytterberg, Steven; Westman, Kerstin; Hoglund, Peter; Harper, Lorraine; Flossmann, Oliver; Luqmani, Raashid; Savage, Caroline; Rasmussen, Niels; de Groot, Kirstin; Tesar, Vladimir; Jayne, David; Merkel, Pater A.; Guillevin, Loic

    2015-01-01

    Objective To analyse the differences between patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) entered into randomised clinical trials (RCTs) and those followed in large observational cohorts. Methods The main characteristics and outcomes of patients with generalised and/or severe GPA or MPA with a five-factor score ≥1 enrolled in the French Vasculitis Study Group (FVSG) or the US-Canadian-based Vasculitis Clinical Research Consortium cohorts were compared to those enrolled in one of 2 FVSG clinical RCTs (WEG91, WEGENT) or 3 European Vasculitis Society clinical trials (CYCLOPS, CYCAZAREM, IMPROVE). Results 657 patients (65.3% with GPA) in RCTs were compared to 437 in cohorts (90.6% with GPA). RCT patients were older at diagnosis than the cohort patients (56.6±13.9 vs. 46.8±17.3 years), had higher Birmingham vasculitis activity score (19.5±9.1 vs. 16.9±7.4), and more frequent kidney disease (84.0% vs. 54.9%) but fewer ear, nose, and throat symptoms (56.8% vs. 72.2%). At 56 months post-diagnosis, mortality and relapse rates, adjusted for age and renal function, were higher for patients with GPA in RCTs vs. cohorts (10.7% vs. 2.5% [p=0.001] and 22.5% vs. 15.6% [p=0.03], respectively) but similar for patients with MPA (6.2% vs. 6.6% [p=0.92] and 16.6% vs. 10.1% [p=0.39], respectively). Conclusion Patients with GPA or MPA in RCTs and those in observational cohorts show important differences that should be remembered when interpreting results based on these study populations. PMID:26016754

  15. Recommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis

    Directory of Open Access Journals (Sweden)

    Alexandre Wagner Silva de Souza

    Full Text Available Abstract The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed, EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

  16. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts

    NARCIS (Netherlands)

    Pagnoux, C.; Carette, S.; Khalidi, N. A.; Walsh, M.; Hiemstra, T. F.; Cuthbertson, D.; Langford, C.; Hoffman, G.; Koening, C. L.; Monach, P. A.; Moreland, L.; Mouthon, L.; Seo, P.; Specks, U.; Ytterbere, S.; Westman, K.; Hoglund, P.; Harper, L.; Flossmann, O.; Luqmani, R.; Savage, C.; Rasmussen, N.; de Groot, K.; Tesar, V.; Jayne, D.; Merkel, P. A.; Guillevin, L.; Stegeman, C. A.

    2015-01-01

    Objective. To analyse the differences between patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) entered into randomised clinical trials (RCTs) and those followed in large observational cohorts. Methods. The main characteristics and outcomes of patients with

  17. ANCA positivity in a patient with infective endocarditis-associated glomerulonephritis: a diagnostic dilemma.

    Science.gov (United States)

    Ghosh, Gopal Chandra; Sharma, Brijesh; Katageri, Bhimarey; Bhardwaj, Minakshi

    2014-09-01

    Glomerulonephritis (GN) is an immunological phenomenon in bacterial endocarditis. These may be pauci-immune/vasculitic GN, post-infective GN, and sub-endothelial membranoproliferative glomerulonephritis. Each type of glomerulonephritis usually occurs in isolation. We report a case of infective endocarditis with dual existence of pauci-immune/vasculitic GN and post infective type of GN at the same time.

  18. Few-layer graphene sheets with embedded gold nanoparticles for electrochemical analysis of adenine

    Directory of Open Access Journals (Sweden)

    Biris AR

    2013-04-01

    Full Text Available Alexandru R Biris,1 Stela Pruneanu,1 Florina Pogacean,1 Mihaela D Lazar,1 Gheorghe Borodi,1 Stefania Ardelean,1 Enkeleda Dervishi,2 Fumiya Watanabe,2 Alexandru S Biris2 1National Institute for Research and Development of Isotopic and Molecular Technologies, Cluj-Napoca, Romania; 2Center for Integrative Nanotechnology Sciences, University of Arkansas at Little Rock, Little Rock, AR, USA Abstract: This work describes the synthesis of few-layer graphene sheets embedded with various amounts of gold nanoparticles (Gr-Au-x over an Aux/MgO catalytic system (where x = 1, 2, or 3 wt%. The sheet-like morphology of the Gr-Au-x nanostructures was confirmed by transmission electron microscopy and high resolution transmission electron microscopy, which also demonstrated that the number of layers within the sheets varied from two to seven. The sample with the highest percentage of gold nanoparticles embedded within the graphitic layers (Gr-Au-3 showed the highest degree of crystallinity. This distinct feature, along with the large number of edge-planes seen in high resolution transmission electron microscopic images, has a crucial effect on the electrocatalytic properties of this material. The reaction yields (40%–50% and the final purity (96%–98% of the Gr-Au-x composites were obtained by thermogravimetric analysis. The Gr-Au-x composites were used to modify platinum substrates and subsequently to detect adenine, one of the DNA bases. For the bare electrode, no oxidation signal was recorded. In contrast, all of the modified electrodes showed a strong electrocatalytic effect, and a clear peak for adenine oxidation was recorded at approximately +1.05 V. The highest increase in the electrochemical signal was obtained using a platinum/Gr-Au-3-modified electrode. In addition, this modified electrode had an exchange current density (I0, obtained from the Tafel plot one order of magnitude higher than that of the bare platinum electrode, which also confirmed that

  19. A Case Report Describing a Rare Presentation of Simultaneous Occurrence of MPO-ANCA-Associated Vasculitis and Rheumatoid Arthritis

    OpenAIRE

    Foray, Nathalie; Hudali, Tamer; Papireddy, Muralidhar; Gao, John

    2016-01-01

    Background. Renal-limited myeloperoxidase vasculitis with simultaneous rheumatoid arthritis is reported as a rare occurrence. Review of literature suggests that most patients had a diagnosis of rheumatoid arthritis for several years prior to presenting with renal failure from myeloperoxidase vasculitis. Case Presentation. A 58-year-old Caucasian male presented to the hospital experiencing malaise, fevers, decreased oral intake, nausea, and vomiting for one week duration. His past medical hist...

  20. Kaposi′s sarcoma with HHV8 infection and ANCA-associated vasculitis in a hemodialysis patient

    Directory of Open Access Journals (Sweden)

    Lilia Ben Fatma

    2013-01-01

    Full Text Available The association between Kaposi′s sarcoma (KS and human herpes virus eight (HHV-8 infection is rarely reported in hemodialysis (HD patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

  1. Muusikamaailm / Priit Kuusk

    Index Scriptorium Estoniae

    Kuusk, Priit, 1938-

    2005-01-01

    Muusikauudiseid maailmast: Festivalidest "Musica Nova" Helsingis, "Maerzmusik" Berliinis; ansambli Haydn Trio tegemistest; Lazar Mermani, Marcello Viotti surmast; uue muusika festivalidest "Archipel", "Klangvisionen", "Merging Voices"

  2. Vaccination of High-Risk Breast Cancer Patients with Carbohydrate Mimicking Peptides

    Science.gov (United States)

    2009-05-01

    animals ANCA006 – CO2 Euthanasia Rev 1 ANCA007 – Rodent Quarantine Rev 1 ANCA008 – Animal Feeding & Bedding ANCA009 – Rodent Ear Marking Rev...participating in it. A clear statement will be made concerning the voluntary nature of her participation and that her decision will have no effect on her...

  3. ECONOMIC AND FINANCIAL ASPECTS OF ACTIVITY IN SC "AGRO ANCA" SRL, RO.IORII DE VEDE, COUNTY TELEORMAN (2011-2012

    Directory of Open Access Journals (Sweden)

    Radu Lucian PÂNZARU

    2014-10-01

    Full Text Available The unit was established in 2008, with the object of activity "permanent cultivation of plants" - NACE Code 011 principal activity "Growing of cereals (except rice, leguminous crops and oil seeds plant". Besides main activity - growing of cereals and other crops, the company may carry on business as a secondary objective: wholesale of grain, seeds feed; Growing of vegetables, horticultural specialties and greenhouse products; cultivation of fruits, nuts, beverage and spice plants; cattle; sheep, goats, horses, asses, mules and donkeys; pig breeding; poultry; Raising of other animals; activities (mixed farming of crops combined with farming of animals; service activities related to agriculture, gardening landscaping (landscape architecture; animal husbandry service activities, except veterinary activities; fisheries; fish; mill products; manufacture of starch and starch products; manufacture of bread, fresh pastry production; road transport of goods; Agricultural machinery and equipment rental; import-export, etc. . The company is part of the Cooperative "VEDEA ROSIORI" - agricultural cooperative whose operating rules are set out in the Statute.

  4. ANCA-Negative Granulomatosis with Polyangiitis Presenting with Hypertrophic Cranial Pachymeningitis, Abducens Nerve Palsy, and Stenosis of the Internal Carotid Artery

    Directory of Open Access Journals (Sweden)

    Shohei Harabuchi

    2017-01-01

    Full Text Available We report a rare case of granulomatosis with polyangiitis (GPA presenting with hypertrophic cranial pachymeningitis (HCP, abducens nerve palsy, and stenosis of the internal carotid artery (ICA. A 59-year-old Japanese man presented with a year history of nasal obstruction and a 2-month history of slight headache. Histopathological examination of the granulomatous mucosa in the ethmoid sinuses resected by endoscopic sinus surgery revealed necrotizing vasculitis with multinucleated giant cells. The patient was diagnosed with the limited form of GPA as a result of the systemic examination. He declined immunosuppressive treatment. Eighteen months after the diagnosis of GPA, he presented with diplopia and severe headache. Though nasal findings indicating GPA were not observed in the nasal cavity, CT scan revealed a lesion of the right sphenoid sinus eroding the bone of the clivus. Gadolinium-enhanced MRI of the brain showed thickening of the dura mater around the right cavernous sinus and clivus. Magnetic resonance angiography and cerebral angiography revealed narrowing at the C5 portion of the ICA. Intravenous methylprednisolone pulse therapy followed by oral prednisolone and cyclophosphamide resolved headache and dramatically improved HCP and stenosis of the ICA.

  5. Agroindustria azucarera y Anca tradicional en el norte plano del Cauca (Colombia). Perspectivas históricas y claves etnográficas

    OpenAIRE

    Jaramillo Marín, Jefferson; Londoño Ortiz, Natalia; Sánchez González, Gina

    2015-01-01

    El artículo reflexiona en clave histórica y etnográfica sobre algunas fricciones y articulaciones entre agroindustria azucarera y finca tradicional en el norte plano del departamento del Cauca desde mediados del siglo xx hasta hoy. La perspectiva histórica permite rastrear la consolidación del modelo agroindustrial azucarero en la zona. La lectura etnográfica describe, desde las narrativas de los pobladores, algunos de los conflictos socioambientales que este modelo genera en la finca tradici...

  6. Vasculites e ANCA – a propósito de um caso clínico

    Directory of Open Access Journals (Sweden)

    Fernando Matos

    1996-01-01

    Full Text Available RESUMO: Apreseotamos o caso clínico de um doente de 68 anos de idade, com glomerulonefrite rapidamente progressiva e hemorragia pulmonar, presença de anticorpos anti-citoplasma dos neutrófilos (padrão perinuclear com especificidade anti-mieloperoxidase, e anticorpos anti-cardiolipina. A histologia renal confirmou o diagnóstico de poliarterite microscópica. A propósito deste caso, são feitas algumas considerações sobre a utilidade dos auto-anticorpos no diagnóstico das diferentes formas de vasculite. ABSRTRACT: We present the case report of a 68 year old man with rapidly progressive glomerulonephritis and pulmonary haemorrhage, with anti-neutrophil cytoplasmic auto-antibodies, perinuclear pattern with myeloperoxidase specifity, and anti-cardiolipin antibodies. Renal histology supported the diagnosis of microscopic polyarteritis. In reference to this case, some considerations regarding the usefullness of autoantibodies in different forms of vasculitis are made. Palavras-chave: Autoanticorpos, Antoantigénios, Endotélio Vascular, Mieloperoxidase, Neutrófilos, Serina proteinase, Vasculites, Key-Words: Autoantibodies, Autoantiges, Endothelium, vascular, Myelope-roxidase, Neutrophils, Serine proteinase, Vasculitis

  7. Algunos Aspectos sobre Manejo, Reproducción y Costos de Producción del Jaguar (panthera anca linné 1875 en Condiciones de Cautividad

    Directory of Open Access Journals (Sweden)

    Vélez S. Gladys

    1979-12-01

    Full Text Available Se observó y analizó el comportamiento y reproducción del Jaguar (Panthera onca en cautividad, por un período de 3 años 1975-1978. Las observaciones sobre su comportamiento, se realizaron en horas diurnas. La pareja conformada por un macho melánico y una hembra manchada, produjo en el período observado un total de 13 crías (4 crías por año, en las cuales la coloración melánica ocurrió en el 61.5% de los casos y la pintada en el 38.5%, la descendencia de la pareja fue predominante de sexo masculino, pero no se encontró que el melanismo estuviera asociado al sexo. Al comparar el costo total de producción, con el precio de la piel animal cazado para el mercado, más el precio de su carne, se encontró que el costo de producción en cautividad es superior en más de diez veces al precio obtenido en el mercado. Como la alimentación representa el 65.8% de los costos de producción, si se reemplaza 50% de la ración a la base de carne, por alimento concentrado, se logra disminuir este costo.

  8. The effects of naturally occurring impurities in rock salt

    Indian Academy of Sciences (India)

    Author Affiliations. Alina-Mihaela Badescu1 Alexandra Saftoiu2. University POLITEHNICA of Bucharest, Splaiul Independen¸tei 313, Sector 6, Bucharest 060042, Romania; IFIN-Horia Hulubei, Str. Reactorului No. 30, P.O. Box MG-6, Bucharest-Magurele, Romania ...

  9. Long-term Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Negative Renal Vasculitis: Cohort Study in Korea.

    Science.gov (United States)

    Lee, Sung Woo; Yu, Mi-Yeon; Baek, Seon Ha; Ahn, Shin-Young; Kim, Sejoong; Na, Ki Young; Chae, Dong-Wan; Chin, Ho Jun

    2016-04-01

    Few studies have reported on the long-term prognosis of anti-neutrophil cytoplasmic antibody (ANCA)-negative renal vasculitis. Between April 2003 and December 2013, 48 patients were diagnosed with renal vasculitis. Their ANCA status was tested using indirect immunofluorescence and enzyme-linked immunosorbent assays. During a median (interquartile range) follow-up duration of 933.5 (257.5-2,079.0) days, 41.7% of patients progressed to end stage renal disease (ESRD) and 43.8% died from any cause. Of 48 patients, 6 and 42 were ANCA-negative and positive, respectively. The rate of ESRD within 3 months was higher in ANCA-negative patients than in ANCA-positive patients (P = 0.038). In Kaplan-Meier survival analysis, ANCA-negative patients showed shorter renal survival than did ANCA-positive patients (log-rank P = 0.033). In univariate Cox-proportional hazard regression analysis, ANCA-negative patients showed increased risk of ESRD, with a hazard ratio 3.190 (95% confidence interval, 1.028-9.895, P = 0.045). However, the effect of ANCA status on renal survival was not statistically significant in multivariate analysis. Finally, ANCA status did not significantly affect patient survival. In conclusion, long-term patient and renal survival of ANCA-negative renal vasculitis patients did not differ from those of ANCA-positive renal vasculitis patients. Therefore, different treatment strategy depending on ANCA status might be unnecessary.

  10. Vasculitis and antineutrophil cytoplasmic autoantibodies associated with propylthiouracil therapy

    NARCIS (Netherlands)

    Dolman, K. M.; Gans, R. O.; Vervaat, T. J.; Zevenbergen, G.; Maingay, D.; Nikkels, R. E.; Donker, A. J.; von dem Borne, A. E.; Goldschmeding, R.

    1993-01-01

    Vasculitis is a rare complication of propylthiouracil therapy. Antineutrophil cytoplasmic antibodies (ANCA) have been described in association with several vasculitic disorders. We report detection of ANCA against human neutrophil elastase, proteinase 3, and myeloperoxidase in serum from six

  11. Evaluation of antineutrophil cytoplasmic antibody seroconversion induced by minocycline, sulfasalazine, or penicillamine

    NARCIS (Netherlands)

    Choi, HK; Slot, MC; Pan, GL; Weissbach, CA; Niles, JL; Merkel, PA

    Objective, Case reports have suggested that minocycline, sulfasalazine, and penicillamine are associated with antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis, This study evaluated ANCA seroconversion due to these agents in serum samples prospectively collected in randomized,

  12. Native and recombinant proteins to analyze auto-antibodies to myeloperoxidase in pauci-immune crescentic glomerulonephritis

    NARCIS (Netherlands)

    Boomsma, MM; Stegeman, CA; Oost-Kort, WW; Kallenberg, CGM; Moguilevsky, N; Limburg, PC; Tervaert, JWC

    2001-01-01

    The prevalence of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) directed against myeloperoxidase (MPO) in pauci-immune necrotizing crescentic glomerulonephritis (NCGN) is dependent on the assay(s) used, We investigated the frequency of MPO-ANCA as detected by different assays for MPO-ANCA in a large

  13. President Ilves ja Simon Peres avasid Tallinna uue sünagoogi / Evely Baum, Riina Leminsky

    Index Scriptorium Estoniae

    Baum, Evely

    2007-01-01

    16. mail 2007 avati pidulikult Tallinna uus juudi sünagoog. Avamistseremoonial osalesid ka Eesti president Toomas Hendrik Ilves ja peaminister Andrus Ansip, Iisraeli asepeaminister Simon Peres, Iisraeli pearabi Yona Metzger, Venemaa pearabi Berl Lazar

  14. Сказка - ложь, да в ней намек... / Виктор Кузнецов

    Index Scriptorium Estoniae

    Кузнецов, Виктор

    2003-01-01

    Nõukogude lastekirjandusest 1930. a-te 2. poolel: Lagin, Lazar. Vanake Hottabõtsh (1938); Volkov, Aleksandr. Smaragdlinna võlur (1939); Tolstoi, Aleksei. Kuldvõtmeke ehk Buratino seiklused (1936); Tshukovski, Kornei. Varastatud päike (1935)

  15. Impressionistide vedurisuitsune Pariis

    Index Scriptorium Estoniae

    1998-01-01

    Maikuu keskpaigani 1998 Musée d'Orsays olnud näitusest 'Manet, Monet & La Gare St. Lazare', mis 14. juunist avati Washingtoni Rahvusgaleriis. Näituse võtmeks oli St. Lazare'i raudteejaam, mida C. Monet on 11 korda maalinud. E. Manet'lt olid näitusel valdavalt Rue St. Petersbourg'i lähikonnast maalitud tööd

  16. Clinical features of usual interstitial pneumonia with anti-neutrophil cytoplasmic antibody in comparison with idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Hosoda, Chiaki; Baba, Tomohisa; Hagiwara, Eri; Ito, Hiroyuki; Matsuo, Norikazu; Kitamura, Hideya; Iwasawa, Tae; Okudela, Koji; Takemura, Tamiko; Ogura, Takashi

    2016-07-01

    Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP). We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy. There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP. ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP. © 2016 Asian Pacific Society of Respirology.

  17. H.S.H. Prince Radu of Hohenzollern-Veringen, Special Representative of the Romanian Government for Integration, Co-operation and Sustainable Development

    CERN Multimedia

    Maximilien Brice

    2002-01-01

    H.S.H. Prince Radu of Hohenzollern-Veringen, Special Representative of the Romanian Government for Integration, Co-operation and Sustainable Development (third from left) with (left to right) Florian Ciolacu (Romanian Mission in Geneva), Cornel Comsa (personal adviser to the Prince), Sorin Ilie (CERN), Calin Alexa (ATLAS), Iosif Legrand (Caltech/CMS), Mihaela Gheata (ALICE), Andrei Gheata (ALICE), and Sorin Zgura (ALICE).

  18. MIHAI EMINESCU ET L’ANALYSE SÉMIOTIQUE DE SON LANGAGE POLITIQUE

    Directory of Open Access Journals (Sweden)

    Lucica BUCULEI (MOROȘAN

    2015-11-01

    Full Text Available Among the studies published on journalistic activity of Romanian poet Mihai Eminescu, we have chosen to highlight the importance of the study of these lesser-known aspects of the artistic life of the poet through a book recently published.This article is a presentation of the book Limbajul eminescian politic. Perspective semiotice by Mihaela Mocanu, as a benchmark for the linguistic and semiotic analysis of the journalistic work of Romanian poet Mihai Eminecu.

  19. Evaluarea unei afaceri in franciza

    OpenAIRE

    Parpandel, Denisa Elena; Belu, Nicoleta; Albici, Mihaela; Tenovici, Cristina; Rizea, Ionela Carmen

    2009-01-01

    Evaluation of a franchise business Parpandel Denisa Elena, Faculty of Management Marketing in Economic Business, Rm. Valcea, “Constantin Brancoveanu” University, Pitesti, Romania Belu Nicoleta, Faculty of Management Marketing in Economic Business, Rm. Valcea, “Constantin Brancoveanu” University, Pitesti, Romania Albici Mihaela, Faculty of Management Marketing in Economic Business, Rm. Valcea, “Constantin Brancoveanu” University, Pitesti, Romania Ţenovici Cristina Otilia, Faculty ...

  20. Pediatric Inflammatory Bowel Disease with Cytoplasmic Staining of Antineutrophil Cytoplasmic Antibodies

    Directory of Open Access Journals (Sweden)

    Omar I. Saadah

    2013-01-01

    Full Text Available Background. It is unusual for the antineutrophil cytoplasmic antibody with cytoplasmic pattern (cANCA to present in patients with inflammatory bowel disease (IBD without vasculitis. The purpose of this study was to describe the occurrence and characteristics of pediatrics IBD with cANCA. Methods. A retrospective review of pediatric IBD associated with cANCA serology in patients from King Abdulaziz University Hospital, Saudi Arabia, between September 2002 and February 2012. Results. Out of 131 patients with IBD screened for cANCAs, cANCA was positive in 7 (5.3% patients of whom 4 had ulcerative colitis and 3 had Crohn's disease. The median age was 8.8 years (2–14.8 years. Six (86% were males. Of the 7 patients, 5 (71% were Saudi Arabians and 2 were of Indian ethnicity. The most common symptoms were diarrhea, abdominal pain, weight loss, and rectal bleeding. None had family history or clinical features suggestive of vasculitis involving renal and respiratory systems. No difference in the disease location or severity was observed between cANCA positive and cANCA negative patients apart from male preponderance in cANCA positive patients. Conclusion. The occurrence of cANCA in pediatric IBD is rare. Apart from male preponderance, there were no peculiar characteristics for the cANCA positive patients.

  1. Antineutrophil Cytoplasmic Antibodies Testing in a Large Cohort of Unselected Greek Patients

    Directory of Open Access Journals (Sweden)

    Konstantinos Tsiveriotis

    2011-01-01

    Full Text Available Objective. To retrospectively evaluate ANCA testing in a cohort of unselected Greek in- and outpatients. Methods. In 10803 consecutive serum samples, ANCA were tested by indirect immunofluorescence (IIF and ELISA. ELISA in inpatients was performed only on IIF positive sera. Results. Low prevalence (6.0% of IIF positive samples was observed. Among these samples, 63.5% presented perinuclear (p-ANCA, 9.3% cytoplasmic (c-ANCA and 27.2% atypical (x-ANCA pattern. 16.1% of p-ANCA were antimyeloperoxidase (anti-MPO positive, whereas 68.3% of c-ANCA were antiproteinase-3 (anti-PR3 positive. Only 17 IIF negative outpatients' samples were ELISA positive. ANCA-associated vasculitides (AAV, connective tissue disorders and gastrointestinal disorders represented 20.5%, 23.9%, and 21.2% of positive results, respectively. AAV patients exhibited higher rates of MPO/PR3 specificity compared to non-AAV (93.8% versus 8%. Conclusions. This first paper on Greek patients supports that screening for ANCA by IIF and confirming positive results by ELISA minimize laboratory charges without sacrificing diagnostic accuracy.

  2. Anti-neutrophil cytoplasmic antibody-associated vasculitis associated with infectious mononucleosis due to primary Epstein-Barr virus infection: report of three cases.

    Science.gov (United States)

    Yamaguchi, Makoto; Yoshioka, Tomoki; Yamakawa, Taishi; Maeda, Matsuyoshi; Shimizu, Hideaki; Fujita, Yoshiro; Maruyama, Shoichi; Ito, Yasuhiko; Matsuo, Seiichi

    2014-02-01

    Although the aetiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis remains unclear, it is generally believed that environmental factors such as infections contribute to its development of ANCA-associated vasculitis. Prior Epstein-Barr virus (EBV) infection is reported to be a trigger of systemic vasculitis. We herein report three cases of ANCA-associated vasculitis presenting with infectious mononucleosis due to primary EBV infection. The causal link between the two pathologies could not be proved, but primary EBV infection may play a role in the initiation or exacerbation of ANCA-associated vasculitis. Future studies are necessary to determine the interaction between these diseases conditions.

  3. Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.

    2011-01-01

    Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 – October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV. PMID:21507463

  4. Relationship among antineutrophil cytoplasmic antibody, blood urea nitrogen and complement in patients with eosinophilic granulomatosis polyangiitis (Churg-Strauss syndrome).

    Science.gov (United States)

    Kawakami, Tamihiro; Kimura, Satoko; Takeuchi, Sora; Soma, Yoshinao

    2013-07-01

    Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by a history of asthma, hypereosinophilia. The prevalence of ANCA in EGPA is less common than in other ANCA-associated vasculitis. Increasing evidence of complement activation in the pathogenesis of ANCA-associated vasculitis has been provided by studies in animal models. We examined EGPA patients with cutaneous manifestations as an initial sign and investigated the correlations among clinical, serological and histopathological findings. We focused on differences among ANCA, blood urea nitrogen and complement levels such as complement 3 (C3), C4 and total complement hemolytic activity (CH50). We retrospectively investigated the records of 22 patients (11 male and 11 female) with EGPA admitted to our hospital from 1997-2012. Ten of the 22 patients (46%) were positive for serum myeloperoxidase (MPO)-ANCA. In contrast, all the patients were negative for serum proteinase 3 ANCA. There was a significantly positive correlation between serum CH50 and C4 levels in patients with EGPA. Serum blood urea nitrogen (BUN) levels differed significantly between MPO-ANCA-positive and -negative patients. Serum CH50 levels were higher in MPO-ANCA-positive patients compared to negative patients. Serum BUN levels were higher in elevated CH50 patients compared to normal and low CH50-negative patients. We propose that positive findings for MPO-ANCA with CH50 high activity may be a risk factor for developing renal insufficiency. Assuming there are correlations between the presence of ANCA and complements, earlier diagnosis based on initial efficacious treatment for EGPA. © 2013 Japanese Dermatological Association.

  5. Quantitation of autoantibodies in systemic autoimmune diseases : clinically useful?

    NARCIS (Netherlands)

    Kallenberg, C. G. M.; Stegeman, C. A.; Bootsma, H.; Biji, M.; Limburg, P. C.

    2006-01-01

    Serial assessment of levels of autoantibodies has been proposed as being clinically useful in certain systemic autoimmune diseases. In particular, attention has been given to anti-dsDNA antibodies in systemic lupus erythematosus (SLE) and ANCA in the ANCA-associated vasculitides (AAV). Much

  6. Anti-proteinase 3 antibodies in diffuse systemic sclerosis (SSc with normotensive renal impairment: is it suggestive for an overlapping between SSc and idiopathic vasculitis?

    Directory of Open Access Journals (Sweden)

    V. Campanella

    2011-09-01

    Full Text Available Objective. To test the prevalence of anti-neutrophil cytoplasmic antibodies (ANCA in systemic sclerosis (SSc and to verify a possible association of ANCA with normotensive renal involvement in SSc. Patients and methods: 51 patients affected by SSc, 35 with diffuse scleroderma (dSSc and 16 with limited scleroderma (lSSc, were tested for ANCA by indirect immunofluorescence (IIF on human ethanol and formalin-acetone-fixed granulocytes (before and after DNase treatment, by conventional enzyme linked immuno-sorbent assay (ELISA and by capture-ELISA. Results. Six out of 51 selected SSc patients had ANCA by IIF (11.7% and five presented a perinuclear/nuclear atypical ANCA pattern. In all cases we only found anti-proteinase3 (aPR3 antibodies. All ANCA positive patients had diffuse form of SSc (17.1%, all were anti-Scl70 positive (aScl70, five patients had proteinuria, three had microscopic haematuria. All ANCA positive patients were normotensive with normal renin plasma levels, the mean erythrocyte sedimentation rate (ESR was higher in this group compared to the other SSc patients. Conclusions. Our study shows that aPR3 is not rare in dSSc. According to the clinical and serological findings and to the recent literature, we can hypothesise that when ANCA are found in SSc, an overlapping of scleroderma with systemic necrotizing vasculitis should be suspected.

  7. Plasma exchange in antineutrophil cytoplasmic antibody-associated vasculitis--a 25-year perspective

    DEFF Research Database (Denmark)

    Szpirt, Wladimir M

    2015-01-01

    Demonstration of a pathogenic role for antineutrophil cytoplasmic antibodies (ANCA) underlies the scientific rationale for plasma exchange (PLEX) in the treatment of ANCA-associated vasculitis (AAV). Most clinical evidence of efficacy concerns the use of PLEX for the recovery of renal function...

  8. A multicentre study to improve clinical interpretation of proteinase-3 and myeloperoxidase anti-neutrophil cytoplasmic antibodies

    DEFF Research Database (Denmark)

    Bossuyt, Xavier; Rasmussen, Niels; van Paassen, Pieter

    2017-01-01

    Objective: The objective of this multicentre study was to improve the clinical interpretation of PR3- and MPO-ANCAs as an adjunct for the diagnosis of ANCA-associated vasculitis (AAV) by defining thresholds and test result intervals based on predefined specificities and by calculating test result...

  9. Disease: H01658 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available toimmune disease characterized by systemic vasculitis. MPA predominantly affects small-calibre blood vessels...irected against proteinase 3 (PR3-ANCA). Not all patients, however, have ANCA. MPA is clinically characterized by small-vessel vascul...itis primarily affecting the kidneys and the lungs but o

  10. Occurrence of antineutrophil cytoplasmic antibodies and associated vasculitis in patients with hyperthyroidism treated with antithyroid drugs : A long-term followup study

    NARCIS (Netherlands)

    Slot, MC; Links, TP; Stegeman, CA; Tervaert, JWC

    2005-01-01

    Objective. To test whether antineutrophil cytoplasmic antibodies (ANCA) and ANCA-associated vasculitis (AAV) are not only induced during treatment with antithyroid drugs, but can also become evident when medication has been ceased, possibly after years. Methods. Patients who visited our hospital for

  11. Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener's granulomatosis : first results from a multicentre study

    NARCIS (Netherlands)

    Csernok, E; Holle, J; Hellmich, B; Willem, J; Tervaert, C; Kallenberg, CGM; Limburg, PC; Niles, J; Pan, GL; Specks, U; Westman, K; Wieslander, J; Gross, WL

    Objective: To evaluate the performance characteristics of direct and capture ELISA for the detection of PR3-ANCA in Wegener's granulomatosis (WG) in international ANCA reference laboratories. Methods: Serum samples were derived from patients with histological and clinical diagnosis of WG (n = 60),

  12. Poor Renal Outcome of Antineutrophil Cytoplasmic Antibody Negative Pauci-immune Glomerulonephritis in Taiwanese

    Directory of Open Access Journals (Sweden)

    Peir-Haur Hung

    2006-01-01

    Conclusion: This study illustrates the necessity for pathologic diagnosis of pauci-immune GN despite ANCA negativity. The poor prognosis associated with ANCA negativity in this study may be partly due to delayed diagnosis since these patients frequently lacked systemic involvement.

  13. Disease: H01655 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available nulomatous inflammation and pauci-immune small- vessel vasculitis of upper and lower respiratory tract and kidneys. The discovery...syndromes. Pr3-ANCA is the predominant autoantibody found in patients with GPA. The discovery of ANCA has ma

  14. Catalase and alpha-enolase : two novel granulocyte autoantigens in inflammatory bowel disease (IBD)

    NARCIS (Netherlands)

    Roozendaal, C; Zhao, MH; Horst, G; Lockwood, CM; Kleibeuker, JH; Limburg, PC; Nelis, GF; Kallenberg, CGM

    1998-01-01

    In IBD, the target antigens of anti-neutrophil cytoplasmic autoantibodies (ANCA) have not been fully identified, which limits the analysis of the diagnostic significance as well as of the possible pathophysiological role of these antibodies. In this study, we identify the target antigens of ANCA in

  15. Catalase and alpha-enolase: two novel granulocyte autoantigens in inflammatory bowel disease (IBD)

    NARCIS (Netherlands)

    Roozendaal, C.; Zhao, M.H.; Lockwood, C.M.; Kleibeuker, Jan; Limburg, Piet; Nelis, G.F.; Kallenberg, Cees; Horst, G.

    1998-01-01

    In IBD, the target antigens of anti-neutrophil cytoplasmic autoantibodies (ANCA) have not been fully identified, which limits the analysis of the diagnostic significance as well as of the possible pathophysiological role of these antibodies. In this study, we identify the target antigens of ANCA in

  16. Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation

    Directory of Open Access Journals (Sweden)

    Semra Ayturk

    2013-01-01

    Full Text Available Propylthiouracil (PTU is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA in few patients with Graves′ disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves′ disease.

  17. Antineutrophil cytoplasm antibody: positivity and clinical correlation.

    Science.gov (United States)

    Martínez Téllez, Goitybell; Torres Rives, Bárbara; Rangel Velázquez, Suchiquil; Sánchez Rodríguez, Vicky; Ramos Ríos, María Antonia; Fuentes Smith, Lisset Evelyn

    2015-01-01

    To determine positivity and clinical correlation of anti-neutrophil cytoplasmic antibodies (ANCA), taking into account the interference of antinuclear antibodies (ANA). A prospective study was conducted in the Laboratory of Immunology of the National Cuban Center of Medical Genetic during one year. Two hounded sixty-seven patients with indication for ANCA determination were included. ANCA and ANA determinations with different cut off points and assays were determined by indirect immunofluorescense. Anti proteinase 3 and antimyeloperoxidase antibodies were determined by ELISA. Most positivity for ANCA was seen in patients with ANCA associated, primary small-vessel vasculitides, rheumatoid arthritis and systemic lupus erythematosus. Presence of ANCA without positivity for proteinase 3 and myeloperoxidase was higher in patients with ANA and little relation was observed between the perinuclear pattern confirmed in formalin and specificity by myeloperoxidase. Highest sensibility and specificity values for vasculitides diagnostic were achieved by ANCA determination using indirect immunofluorescense with a cut off 1/80 and confirming antigenic specificities with ELISA. ANCA can be present in a great number of chronic inflammatory or autoimmune disorders in the population studied. This determination using indirect immunofluorescence and following by ELISA had a great value for vasculitis diagnosis. Anti mieloperoxidasa assay has a higher utility than the formalin assay when ANA is present. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  18. Marfan syndrome with antineutrophil cytoplasmic antibody-associated systemic vasculitis presenting as severe anaemia and haematuria after the Bentall procedure.

    Science.gov (United States)

    Sijia, Li; Shuangxin, Liu; Wei, Shi; Yanhai, Cui

    2013-08-01

    One month previously, a 28-year old male underwent an emergency modified Bentall procedure because of Marfan syndrome with acute aortic dissection Stanford Class A. Computed tomography of the chest did not reveal severe graft stenosis of the anastomosis. To explore the cause of anaemia, renal dysfunction and macroscopic haematuria, the patient was tested for antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV). Antimyeloperoxidase antibodies (MPO)-ANCA and antiproteinase 3 antibodies (PR3)-ANCA were strongly positive. Corticosteroid therapy was applied, followed by cyclophosphamide and azathioprine. In response to treatment, the MPO-ANCA and PR3-ANCA levels gradually decreased, proteinuria was alleviated and haemoglobin levels returned to normal after 6 months. This is the first report to highlight haemolytic anaemia and AASV with Marfan syndrome after surgery for aortic dissection.

  19. Churg-Strauss syndrome

    Directory of Open Access Journals (Sweden)

    Ilić Tatjana

    2002-01-01

    Full Text Available Churg-Strauss syndrome (CSS is medium blood vessels vasculitis with predilection for lungs in patients with bronchial asthma, chronic eosinophilic pneumonia and positive ANCA in the sera in 55-67%. This is a case report of a 60 years old female patient with bronchial asthma, peripheral pulmonary infiltrations, blood eosinophilia, xerophtalmia, tachycardia, chronic rhino sinusitis, polyneuropathia and negative immunological tests: CIC (PEG, CRYO, ANA (IIF, RF (agglutination and ANCA (IIF: pANCA and cANCA; ELISA: proteinase 3, lactoferrin, myeloperoxidase, elastase, cathepsin G. Eosinophilic infiltrates in the tissues tested by skin and salivary gland biopsies were not found. The patient had fulfilled five clinical diagnostic criteria and responded well to immunosuppressive therapy, so this case could be classified as the ANCA negative angiitis and granulomatous of CSS type.

  20. Estudio longitudinal de anticuerpos anticitoplasma de neutrófilos en pacientes con anemia drepanocítica Longitudinal study of antineutrophil cytoplasmic antibodies in patients with sickle cell anemia

    Directory of Open Access Journals (Sweden)

    Ana María Guerreiro Hernández

    2000-08-01

    Full Text Available Se realizó un estudio longitudinal para detectar anticuerpos anticitoplasma de neutrófilos (ANCA en 13 pacientes con anemia drepanocítica en crisis vasooclusiva y en estado basal, mediante un método de inmunofluorescencia indirecta. Del total de 34 muestras de suero obtenidas, 16 fueron en crisis vasooclusiva y en 12 de ellas, correspondientes a 10 pacientes, se demostró la presencia de p-ANCA. En el resto de las muestras en crisis vasooclusiva y en estado basal no se observó la presencia de p-ANCA o c-ANCA. Los resultados obtenidos sugieren la posible participación de los p-ANCA en el daño isquémico, así como la importancia de su medición en el diagnóstico de las crisis vasooclusivas (CVO en los pacientes con anemia drepanocítica (ADA longitudinal study was made to detect antineutrophil cytoplasmic antibodies (ANCA in 13 patients with sickle cell anemia in vasocclusive crisis and basal state by using an indirect immunofluorescence method. Of 34 serum samples, 16 were in vasocclusive crisis and 12 of them corresponding to 10 patients revealed the presence of p-ANCA. Neither p-ANCA nor c-ANCA was observed in the rest of the samples taken in vasoclussive crisis and in basal state. The results achieved signaled a possible involvement of p-ANCA in ischemic damage as well as the importance of their measurement in the diagnosis of vasocclusive crisis in patients with sickle cell anemia

  1. Sano, kuracado, higieno (health, medicine, hygiene): ophthalmologists and Esperanto.

    Science.gov (United States)

    Ohry, Avi

    2014-01-01

    Historically, some ophthalmologists were closely associated with the constructed language Esperanto. These include the founder of the language Ludwig Lazar Zamenhof (1859-1917), his son Leon, Max Talmud (1869-1941), Kazimierz Bein (1872-1959), and Louis Emile Javal (1839-1907).This paper considers the role of these ophtalmologists who attempted to create international languages, whether oral, written or musical.

  2. Editorial

    Directory of Open Access Journals (Sweden)

    Bengisu Bayrak

    2018-04-01

    Full Text Available Volume 6.2 (2017 includes the articles by Hasan Gürkan, Bahar Muratoğlu Pehlivan & Gül Esra Atalay, Andrew Ali Ibbi, Iqbal Shailo, Alon Lazar & Tal Litvak Hirsch, Elloit Cardozo, Floribert Patrick C. Endong, Olugbenga Elegbe, Volkan Yücel & Ziya Toprak, Aslı Daldal, Funda Mardar Kara & Şakir Eşitti.

  3. Postcolonial theory as a hermeneutical tool for Biblical reading

    African Journals Online (AJOL)

    p1243322

    this field of study is increasingly attracting greater interest from feminist and ... 1 Dr Lazare S Rukundwa is the Executive Secretary of Eben-Ezer Ministry International in the .... argues that since biblical texts are products of colonial experiences, a ... developed in a foreign context cannot effectively respond to the socio-.

  4. Film Review Jerusalema (2008) | Hees | Journal of the Musical Arts ...

    African Journals Online (AJOL)

    Original Music: Alan Ari Lazar. Cast: Rapulana Seiphemo, Ronnie Nyakale, Jeffrey Zekele, Kenneth Nkosi, helley Meskin, Robert Hobbs, Eugene Khumbanyiwa, Jafta Mamabolo, Motlatsi Mahloko. Approx 120 min. Distributor: Next Video, Fountain Grove, 5 Second Street, Hyde Park. . Journal of the Musical Arts in Africa ...

  5. Kas Venemaa ülemrabil on õigus?

    Index Scriptorium Estoniae

    2004-01-01

    Ilmunud ka: Severnoje Poberezhje, 20. juuli 2004, lk. 2. Venemaa ülemrabi Berl Lazar pöördus Euroopa Komisjoni presidendi Romano Prodi poole üleskutsega anda hinnang Eesti võimude tegevusele, mis rabi meelest soosib kohalikke natse. Küsimusele vastab Ida-Viru juudi kogukonna juht Aleksandr Dusman

  6. В Таллинне открылась новая синагога / Ксения Репсон

    Index Scriptorium Estoniae

    Репсон, Ксения

    2007-01-01

    Tallinnas Karu tänaval avati 16. mail 2007 uus juudi sünagoog, avamistseremoonial viibisid Iisraeli asepeaminister Shimon Peres, Eesti president Toomas Hendrik Ilves, peaminister Andrus Ansip, Venemaa pearabi Berl Lazar, Iisraeli pearabi Yona Metzger ja sünagoogi suurrahastaja Aleksander Bronštein. President Toomas Hendrik Ilves viitas oma kõnes paralleelidele eestlaste ja juutide ajaloos

  7. The new synagogue has given the Jewish community something to sing and dance about / Rein Sikk

    Index Scriptorium Estoniae

    Sikk, Rein, 1961-

    2007-01-01

    Tallinnas Karu tänaval avati 16. mail 2007 uus juudi sünagoog, avamistseremoonial viibisid Iisraeli asepeaminister Shimon Peres, Eesti president Toomas Hendrik Ilves, peaminister Andrus Ansip, Venemaa pearabi Berl Lazar, Iisraeli pearabi Yona Metzger ja sünagoogi suurrahastaja Aleksander Bronštein. President Toomas Hendrik Ilves viitas oma kõnes paralleelidele eestlaste ja juutide ajaloos

  8. School-University Partnerships and Physical Education Teacher Education Student Learning: A Fruitful Division of Labour?

    Science.gov (United States)

    Chambers, Fiona; Armour, Kathleen

    2012-01-01

    Tikunoff, Ward & Lazar (1980) argue that parity or "equality of status" (p.12) is one of the most important ingredients of successful collaboration. During the placement of students on teaching practice (TP), therefore, it can be argued that school and university personnel should be equal partners if the collaboration is to be…

  9. Quantitative trait loci affecting phenotypic variation in the vacuolated lens mouse mutant, a multigenic mouse model of neural tube defects

    NARCIS (Netherlands)

    Korstanje, Ron; Desai, Jigar; Lazar, Gloria; King, Benjamin; Rollins, Jarod; Spurr, Melissa; Joseph, Jamie; Kadambi, Sindhuja; Li, Yang; Cherry, Allison; Matteson, Paul G.; Paigen, Beverly; Millonig, James H.

    Korstanje R, Desai J, Lazar G, King B, Rollins J, Spurr M, Joseph J, Kadambi S, Li Y, Cherry A, Matteson PG, Paigen B, Millonig JH. Quantitative trait loci affecting phenotypic variation in the vacuolated lens mouse mutant, a multigenic mouse model of neural tube defects. Physiol Genomics 35:

  10. IN MEMORIAM ION VATAMANU

    Directory of Open Access Journals (Sweden)

    S.P. Palii

    2012-12-01

    Full Text Available A dreamer in his creative solitude, an objective and lucid analyst of history and contemporaneity, an energetic and decisive leader with an uncanny ability for crisis management – all these describe Ion Vatamanu. His wife Elena and daughters Mihaela, Mariana, Leontina treasure a personal universe in which the magical spark of Ion Vatamanu’s love and joy of life meld the everyday in and out of poetry. Ion Vatamanu’s instantaneous connection to the audiences and deeply felt words still touch the hearts of his many colleagues and friends. Downloads: 2

  11. Uudiseid maailmast / Nele-Eva Steinfeld

    Index Scriptorium Estoniae

    Steinfeld, Nele-Eva

    2012-01-01

    Lühisõnumeid muusikamaailmast: Paavo Järvist saab Tokyo NHK sümfooniaorkestri peadirigent. Suri Dietrich Fischer-Dieskau. Avastati Antonio Vivaldi uusi teoseid. Glenn Gouldi klaver jõudis Ottawa Rahvuslikku Kunstide Keskusesse. Sbeliuse noodistustarkvara peakorter Suurbritannias suleti. Viini Riigiooperi rekordiliselt kasumlik hooaeg. Bayreuthi markkrahvi ooperimaja arvati UNESCO maailmapärandi nimistusse. Erkki-Sven Tüüri ooperi "Wallenberg" uuslavastus Karlsruhes. Pianist Mihkel Poll andis kontserdi Wigmore Hallis. Rahvusvaheline heliloojate rostrum Stockholmis. Suri viiuldaja Ruggiero Ricci. Lahkus pianist Mihaela Ursuleasa

  12. Anti-neutrophil cytoplasmic antibodies stimulate release of neutrophil microparticles.

    LENUS (Irish Health Repository)

    Hong, Ying

    2012-01-01

    The mechanisms by which anti-neutrophil cytoplasmic antibodies (ANCAs) may contribute to the pathogenesis of ANCA-associated vasculitis are not well understood. In this study, both polyclonal ANCAs isolated from patients and chimeric proteinase 3-ANCA induced the release of neutrophil microparticles from primed neutrophils. These microparticles expressed a variety of markers, including the ANCA autoantigens proteinase 3 and myeloperoxidase. They bound endothelial cells via a CD18-mediated mechanism and induced an increase in endothelial intercellular adhesion molecule-1 expression, production of endothelial reactive oxygen species, and release of endothelial IL-6 and IL-8. Removal of the neutrophil microparticles by filtration or inhibition of reactive oxygen species production with antioxidants abolished microparticle-mediated endothelial activation. In addition, these microparticles promoted the generation of thrombin. In vivo, we detected more neutrophil microparticles in the plasma of children with ANCA-associated vasculitis compared with that in healthy controls or those with inactive vasculitis. Taken together, these results support a role for neutrophil microparticles in the pathogenesis of ANCA-associated vasculitis, potentially providing a target for future therapeutics.

  13. Antineutrophil Cytoplasmic Antibodies Associated With Infective Endocarditis

    Science.gov (United States)

    Langlois, Vincent; Lesourd, Anais; Girszyn, Nicolas; Ménard, Jean-Francois; Levesque, Hervé; Caron, Francois; Marie, Isabelle

    2016-01-01

    Abstract To determine the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with infective endocarditis (IE) in internal medicine; and to compare clinical and biochemical features and outcome between patients exhibiting IE with and without ANCA. Fifty consecutive patients with IE underwent ANCA testing. The medical records of these patients were reviewed. Of the 50 patients with IE, 12 exhibited ANCA (24%). ANCA-positive patients with IE exhibited: longer duration between the onset of first symptoms and IE diagnosis (P = 0.02); and more frequently: weight loss (P = 0.017) and renal impairment (P = 0.08), lower levels of C-reactive protein (P = 0.0009) and serum albumin (P = 0.0032), involvement of both aortic and mitral valves (P = 0.009), and longer hospital stay (P = 0.016). Under multivariate analysis, significant factors for ANCA-associated IE were: longer hospital stay (P = 0.004), lower level of serum albumin (P = 0.02), and multiple valve involvement (P = 0.04). Mortality rate was 25% in ANCA patients; death was because of IE complications in all these patients. Our study identifies a high prevalence of ANCA in unselected patients with IE in internal medicine (24%). Our findings further underscore that ANCA may be associated with a subacute form of IE leading to multiple valve involvement and more frequent renal impairment. Because death was due to IE complications in all patients, our data suggest that aggressive therapy may be required to improve such patients’ outcome. PMID:26817911

  14. Hydralazine-induced pauci-immune glomerulonephritis: intriguing case series with misleading diagnoses

    Directory of Open Access Journals (Sweden)

    Faizan Babar

    2016-04-01

    Full Text Available Hydralazine has been used since the 1950s for the management of hypertension. Evidence for hydralazine-associated vasculitis dates to pre-ANCA (antineutrophil cytoplasmic antibodies era. This abstract describes two cases of ANCA-positive pauci-immune glomerulonephritis (GN in challenging scenarios where diagnosis was misconstrued. A comprehensive literature review was done to understand the pathogenesis of drug-induced pauci-immune GN. We have described key diagnostic features that are helpful in distinguishing idiopathic ANCA vasculitis from drug-induced vasculitis. Additionally, we have also described different treatments meant to provide therapy options with the least side effects.

  15. Implications of Antiphospholipid and Antineutrophilic Cytoplasmic Antibodies in the Context of Postinfectious Glomerulonephritis

    Directory of Open Access Journals (Sweden)

    Daniel Leifer

    2017-01-01

    Full Text Available While antineutrophil cytoplasmic antibody (ANCA positivity has been documented in some patients with postinfectious glomerulonephritis (PIGN and is associated with more severe disease, antiphospholipid antibodies (APA are not known to be a common occurrence. We describe a child with severe acute kidney injury who was noted to have prolonged positivity of both ANCA and APA; a renal biopsy showed noncrescentic immune complex mediated glomerulonephritis with subepithelial deposits compatible with PIGN. He recovered without maintenance immunosuppressive therapy and at last follow-up had normal renal function. We discuss the cooccurrence and implications of ANCA and APA in children with PIGN.

  16. [A case of mixed connective tissue disease positive for proteinase 3 antineutrophil cytoplasmic antibody in a patient with slowly progressive type 1 diabetes mellitus and chronic thyroiditis].

    Science.gov (United States)

    Michitsuji, Tohru; Horai, Yoshiro; Sako, Ayaka; Asano, Taro; Iwanaga, Nozomi; Izumi, Yasumori; Kawakami, Atsushi

    2017-01-01

      A female in her sixties with slowly progressive type 1 diabetes mellitus (SPT1DM) and chronic thyroiditis was referred to our rheumatology department with swelling in her fingers. A prominent atherosclerotic lesion was revealed upon brain magnetic resonance imaging, and she was found to have mixed connective tissue disease (MCTD) positive for proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA). This rare case of MCTD accompanying SPT1DM and PR3-ANCA suggested that a synergy between MCTD and PR3-ANCA triggers atherosclerosis.

  17. Disease: H01688 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA)....lectron microscopy. Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the

  18. Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis

    NARCIS (Netherlands)

    Rutgers, Abraham; Kallenberg, Cornelis

    Purpose of review Induction treatment of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) is not always successful and nonresponding patients are considered refractory. Recent findings Refractory disease should be subdefined to the treatment that was received.

  19. Coagulation Factors Test

    Science.gov (United States)

    ... Blood Testing Alpha-1 Antitrypsin Alpha-fetoprotein (AFP) Tumor Marker AMAS Aminoglycoside Antibiotics Ammonia Amniocentesis Amylase ANCA/MPO/ ... Beta-2 Microglobulin Kidney Disease Beta-2 Microglobulin Tumor Marker Bicarbonate (Total CO2) Bilirubin Blood Culture Blood Gases ...

  20. ASO: Antistreptolysin O titer

    Science.gov (United States)

    ... Phosphatase (ALP) Allergy Blood Testing Alpha-1 Antitrypsin Alpha-fetoprotein (AFP) Tumor Marker AMAS Aminoglycoside Antibiotics Ammonia Amniocentesis Amylase ANCA/MPO/PR3 Antibodies Androstenedione Angiotensin-Converting Enzyme ( ...

  1. Pauci-immune necrotizing glomerulonephritis

    NARCIS (Netherlands)

    Rutgers, Abraham; Sanders, Jan S F; Stegeman, Coen A; Kallenberg, Cees G M

    Pauci-immune necrotizing glomerulonephritis is the most frequent cause of rapidly progressive glomerulonephritis and, in most cases, is associated with antineutrophil cytoplasmic antibodies (ANCA). It is either the renal manifestation of Wegener's granulomatosis, microscopic polyangiitis of

  2. Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    DEFF Research Database (Denmark)

    Morishita, Kimberly A; Moorthy, Lakshmi N; Lubieniecka, Joanna M

    2017-01-01

    diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission...

  3. [Update Churg-Strauss syndrome].

    Science.gov (United States)

    Moosig, F; Hellmich, B

    2012-11-01

    The Churg-Strauss syndrome (CSS) is the rarest subtype of the so-called anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and has the lowest frequency of ANCA-positivity (around 30%). In addition to asthma and blood eosinophilia, CSS is characterized by end-organ damage, which can be caused by either vasculitis and/or tissue infiltration of eosinophilic granulocytes. The CSS shares many etiological and clinical features of other hypereosinophilic syndromes. Recently, a distinct genetic background could be demonstrated for both the ANCA-positive and ANCA-negative subtypes of CSS as compared to the other two forms of AAV. Among other cytokines, interleukin-5 (IL-5) could be identified as a key mediator of eosinophilia. Therefore, recent clinical trials in CSS aimed to target IL-5. Outside of clinical trials, treatment of CSS is adapted to disease stage and activity, as recommended for other types of AAV.

  4. A case of small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    Madhulika Mahashabde

    2014-01-01

    We are reporting a case of un-specified small vessel vasculitis, which was diagnosed on the basis of positive perinuclear anti neutrophil cytoplasmic antibodies (ANCA P MPO done by Enzyme Linked Immunosorbent Assay (ELISA.

  5. Central nervous system vasculitis caused by propylthiouracil therapy: a case report and literature review.

    Science.gov (United States)

    Vanek, C; Samuels, M H

    2005-01-01

    Antineutrophil cytoplasmic antibodies (ANCA) are associated with vasculitis, including vasculitis induced by drugs such as the thionamides. The affected organ systems in thionamide-induced vasculitis have been primarily renal, musculoskeletal, and dermatologic. We describe the first case of thionamide-induced central nervous system vasculitis presenting as confusion, with complete resolution after discontinuation of propylthiouracil. We review the literature and summarize 42 additional cases of thionamide-induced ANCA-positive vasculitis since 1992. Propylthiouracil was responsible in 93% of cases and the predominant ANCA pattern on immunofluorescent staining was perinuclear (p-ANCA). Clinical improvement occurred after drug discontinuation in 93%, steroid therapy was used in some cases. The mean duration of treatment with thionamides was 35 months prior to presentation. Long-term medical treatment with thionamides for hyperthyroidism may increase the risk of this severe side effect.

  6. Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

    Science.gov (United States)

    Thiel, Jens; Hässler, Fabian; Salzer, Ulrich; Voll, Reinhard E; Venhoff, Nils

    2013-09-24

    Eosinophilic granulomatosis with polyangiitis (EGPA) is part of antineutrophil cytoplasmic antibodies (ANCAs)-associated vasculitides. In EGPA small-vessel vasculitis is associated with eosinophilia and asthma. About 40% of EGPA patients are ANCA-positive, suggesting a role for B cells in the pathogenesis of EGPA. B cell-depleting therapy with rituximab (RTX) can be effective in ANCA-positive EGPA, but very few patients have been published to date. The role of RTX in the treatment of ANCA-negative EGPA is unclear. We report a single-center cohort of patients with eosinophilic granulomatosis with polyangiitis. Of these patients, nine (six ANCA-positive, three ANCA-negative) had been treated with RTX for relapsing or refractory disease on standard immunosuppressive treatment. In a retrospective analysis, data on treatment response, frequency of relapses, adverse events, and peripheral B-cell reconstitution were evaluated. Furthermore, serum immunoglobulin concentrations, ANCA status, and peripheral B cell subpopulations were assessed after RTX treatment. All patients had high disease activity before RTX treatment. At presentation 3 months after RTX therapy, all ANCA-positive and ANCA-negative patients had responded to RTX, with one patient being in complete remission, and eight patients being in partial remission. After a mean follow-up of 9 months, C-reactive protein concentrations had normalized, eosinophils had significantly decreased, and prednisone had been tapered in all patients. In all patients, RTX therapy was combined with a standard immunosuppressive therapy. Within the 9-month observation period, no relapse was recorded. Three patients were preemptively retreated with RTX, and during the median follow-up time of 3 years, no relapse occurred in these patients. During the follow-up of 13 patient-years, five minor but no major infections were recorded. In our analysis on nine patients with EGPA resistant to standard therapy, rituximab proved to be an

  7. A case of propylthiouracil-induced antineutrophilic cytoplasmic antibody-positive vasculitis successfully treated with radioactive iodine

    Directory of Open Access Journals (Sweden)

    C. Bes

    2013-07-01

    Full Text Available Antineutrophilic cytoplasmic antibody (ANCA associated vasculitis is one of the rare complications of propylthiouracil treatment. Having a variable clinical spectrum, it may be presented with both skin limited vasculitis and life-threatening systemic vasculitis. In this study, we present a case that developed ANCA-positive vasculitis with skin and kidney involvement (hematuria and proteinuria six months after propylthiouracil treatment was initiated for toxic nodular goiter. Proteinuria recovered dramatically subsequent to radioactive iodine treatment following ceasing the drug.

  8. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

    OpenAIRE

    Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophili...

  9. Anti-Myeloperoxidase Antibodies Associate with Future Proliferative Lupus Nephritis

    Directory of Open Access Journals (Sweden)

    S. W. Olson

    2017-01-01

    Full Text Available Background. The subclinical pathophysiology of proliferative lupus nephritis (PLN has not been fully elucidated. Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA is associated with PLN, but prediagnostic levels have not been reported. Methods. We performed a retrospective case-control Department of Defense Serum Repository (DoDSR study comparing MPO-ANCA levels in longitudinal prediagnostic serum samples for 23 biopsy confirmed proliferative lupus nephritis (PLN patients to DoDSR identified age, sex, race, and age of serum matched healthy and SLE without LN disease controls. We also compared the temporal relationship of MPO-ANCA to anti-double stranded DNA antibodies (dsDNAab. Results. A greater proportion of PLN patients had prediagnostic MPO-ANCA levels above ≥3 U/mL and ≥6 U/mL compared to SLE without LN (91% versus 43%, p<0.001; 57% versus 5%, p<0.001, resp.. In subgroup analysis, the MPO-ANCA threshold of ≥3 U/mL was significant at <1 year (88% versus 39%, p=0.007 and 1–4 years (87% versus 38%, p=0.009 prior to diagnosis. Statistically significant subclinical MPO-ANCA levels (≥3 U/mL occurred prior to statistically significant dsDNAab ≥ 3 IU/ml (89% versus 11%, p=0.003. Conclusions. Subclinical MPO-ANCA levels could distinguish future PLN from SLE without LN. MPO-ANCA manifests prior to clinical disease and subclinical dsDNAab to suggest that it may contribute directly to PLN pathogenicity.

  10. Pulmonary CT findings of Wegener's granulomatosis (WG). Follow-up images and pathology

    International Nuclear Information System (INIS)

    Uezono, Haruka; Noma, Satoshi; Sakamoto, Ryo

    2009-01-01

    We reviewed 16 cases of Wegener's granulomatosis (WG) with regard to CT findings, PR3-ANCA, and clinical courses. Because of PR3-ANCA, atypical manifestations of WG could have been diagnosed, including tiny nodules without symptoms and self-remissions. Radiologists should be aware of these atypical manifestations of WG. Seeing multiple nodules in lung, WG should be included in the differential diagnosis and extrapulmonary manifestation of WG should be evaluated. (author)

  11. Systemic vasculitis and the lung.

    Science.gov (United States)

    Talarico, Rosaria; Barsotti, Simone; Elefante, Elena; Baldini, Chiara; Tani, Chiara; Mosca, Marta

    2017-01-01

    The purpose of this review is to provide a critical analysis of the recent literature on this topic, with particular focus on the most relevant studies published over the last year. Many studies are published every year on the diagnosis, pathogenesis and treatment of pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). The main subjects covered by this article are the pathogenesis, diagnosis and clinical aspects of lung involvement in ANCA-associated vasculitis and non-ANCA-associated vasculitis. Lung involvement is a common feature in systemic vasculitis. The lungs are one of the most frequently involved organs in systemic vasculitis. In order to provide an update on the recent advances in the pathogenesis, clinical features and novel treatments of lung involvement in systemic vasculitis, a systematic MedLine search has been performed.Most of the data analyzed have confirmed that lung involvement seems to develop more frequently in patients with myeloperoxidase-ANCA-positive AAV, mainly in those with a diagnosis of microscopic polyangiitis (MPA), compared with patients with proteinase 3 ANCA-positive AAV. Moreover, among non-ANCA-associated vasculitis lung involvement may represent a worrying complication of the disease, mainly when associated with vascular involvement.

  12. LA GLOBALITZACIÓ I ELS SISTEMES FEDERALS

    Directory of Open Access Journals (Sweden)

    Xavier Arbós Marín

    2005-10-01

    Full Text Available This article is a revised analysis ofthe work by Lazar, Telford andWatts (2003 The Impact of Globaland Regional Integration on FederalSystems. A ComparativeAnalysis, Harvey Lazar, HamishTelford and Ronald L. Watts (eds.:The Impact of Global and RegionalIntegration on Federal Systems.A Comparative Analysis,Montreal and Kingston: McGill-Queen’s University Press, 2003.The article’s starting point is thebook’s mainthesis, that is, an analysisof the consequences that suchdifferent integration processescan have both on the institutionalstructures and on the collectiveidentities of federal systems; inthis sense and in the light of thesechanging pressures on the natureof federalism, the article raises aquestion: to what extent federalismand federal arrangementswould remain a reference for carryingout institutional reforms aswell as for a politically and sociallypracticable system.

  13. Radiation-Induced Germ Cell Mutations-Their Detection and Modification.

    Science.gov (United States)

    1987-06-30

    the zygote before or soon after implantation. Boue et. al (13) estimate that 66% of all early abortuses have chromosomal anomalies, and the...Ann. Hum. Genet. 36: 185-194, 1972. 6. Lazar, P., S. Gueguen, J. Boue and A. Boue . Epidemiologie des avortements 0 spontanes precoces: A propos de 1...469 avortements caryotypes, in: A. Boue * and C. Thibault (eds.), Les Accidents Chromosomniques de la Reproduction, .INSERM, Paris, 1973, pp. 319-331. 7

  14. Towards a Possible Therapy for Diabetes Complications

    Science.gov (United States)

    2012-10-01

    pancreatic beta cells. Type 2 diabetes (T2D) patients are, instead, afflicted by an heterogeneous set of sub- syndromes characterized by peripheral...military, their families , and retired military personnel. Finding a cure to T1D will provide significant healthcare savings and improved patients’ well...Circulation 2000: 101: 235–238. 60. Lehrke M, Lazar MA. The many faces of PPARgamma. Cell 2005: 123: 993–999. 61. Duan SZ, Usher MG, Mortensen RM. Peroxi- some

  15. A Biophysico-Computational Perspective of Breast Cancer Pathogenesis and Treatment Response

    Science.gov (United States)

    2011-03-01

    with MyoD. Mol Endocrinol 13, 1155-1168 (1999). 35 Guenther, M. G., Barak , O. & Lazar, M. A. The SMRT and N-CoR corepressors are activating cofactors...embryonic stem cells.  J Biol Chem. 2001;276(28):25647–25650.   17.  Rosen  EM, Fan S, Pestell RG, Goldberg ID. BRCA1  gene in breast cancer. J Cell

  16. Inhibitors for Androgen Receptor Activation Surfaces

    Science.gov (United States)

    2006-09-01

    during growth and development, as well as general metabolism (4–6). Abnormal levels of T3 are responsible for medical condi- tions such as obesity, high...1142 (2001). 5. J. Malm, Thyroid hormone ligands and metabolic diseases. Curr. Pharm. Des. 10, 3525�3532 (2004). 6. J. Zhang, M. A. Lazar, The mechanism...Panidis, Resistin levels in hyperthyroid patients before and after restoration of thyroid function: Relationship with body weight and body

  17. Assessment of predictivity of volatile organic compounds carcinogenicity and mutagenicity by freeware in silico models.

    Science.gov (United States)

    Guerra, Lília Ribeiro; de Souza, Alessandra Mendonça Teles; Côrtes, Juliana Alves; Lione, Viviane de Oliveira Freitas; Castro, Helena Carla; Alves, Gutemberg Gomes

    2017-12-01

    The application of in silico methods is increasing on toxicological risk prediction for human and environmental health. This work aimed to evaluate the performance of three in silico freeware models (OSIRIS v.2.0, LAZAR, and Toxtree) on the prediction of carcinogenicity and mutagenicity of thirty-eight volatile organic compounds (VOC) related to chemical risk assessment for occupational exposure. Theoretical data were compared with assessments available in international databases. Confusion matrices and ROC curves were used to evaluate the sensitivity, specificity, and accuracy of each model. All three models (OSIRIS, LAZAR and Toxtree) were able to identify VOC with a potential carcinogenicity or mutagenicity risk for humans, however presenting differences concerning the specificity, sensitivity, and accuracy. The best predictive performances were found for OSIRIS and LAZAR for carcinogenicity and OSIRIS for mutagenicity, as these softwares presented a combination of negative predictive power and lower risk of false positives (high specificity) for those endpoints. The heterogeneity of results found with different softwares reinforce the importance of using a combination of in silico models to occupational toxicological risk assessment. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Sphingosine-1-phosphate (S1P) enhances glomerular endothelial cells activation mediated by anti-myeloperoxidase antibody-positive IgG.

    Science.gov (United States)

    Sun, Xiao-Jing; Chen, Min; Zhao, Ming-Hui

    2018-03-01

    Cumulating evidences suggested an important role of sphingosine-1-phosphate (S1P) and its receptors in regulating endothelial barrier integrity. Our previous study revealed that the circulating S1P levels and renal expression of S1PRs correlated with disease activity and renal damage in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This study investigated the role of S1P and its receptors in myeloperoxidase (MPO)-ANCA-positive IgG-mediated glomerular endothelial cell (GEnC) activation. The effect of S1P on morphological alteration of GEnCs in the presence of MPO-ANCA-positive IgG was observed. Permeability assay was performed to determine endothelial monolayer activation in quantity. Both membrane-bound and soluble ICAM-1 and VCAM-1 levels were measured. Furthermore, antagonists and/or agonists of various S1PRs were employed to determine the role of different S1PRs. S1P enhanced MPO-ANCA-positive IgG-induced disruption of tight junction and disorganization of cytoskeleton in GEnCs. S1P induced further increase in monolayer permeability of GEnC monolayers in the presence of MPO-ANCA-positive IgG. S1P enhanced MPO-ANCA-positive IgG-induced membrane-bound and soluble ICAM-1/VCAM-1 up-regulation of GEnCs. Soluble ICAM-1 levels in the supernatants of GEnCs stimulated by S1P and MPO-ANCA-positive IgG increased upon pre-incubation of S1PR1 antagonist, while pre-incubation of GEnCs with the S1PR1 agonist down-regulated sICAM-1 level. Blocking S1PR2-4 reduced sICAM-1 levels in the supernatants of GEnCs stimulated by S1P and MPO-ANCA-positive IgG. Pre-incubation with S1PR5 agonist could increase sICAM-1 level in the supernatants of GEnC stimulated by S1P and MPO-ANCA-positive IgG. S1P can enhance MPO-ANCA-positive IgG-mediated GEnC activation through S1PR2-5. © 2017 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

  19. Antineutrophil cytoplasmic autoantibodies and myeloperoxidase autoantibodies in clinical expression of Churg-Strauss syndrome.

    Science.gov (United States)

    Healy, Bridget; Bibby, Susan; Steele, Richard; Weatherall, Mark; Nelson, Harold; Beasley, Richard

    2013-02-01

    The clinical significance of antineutrophil cytoplasmic antibodies (ANCAs) in the phenotypic expression of Churg-Strauss syndrome (CSS) is uncertain. We sought to investigate the relationship between ANCA status and the clinical expression of CSS in a case series derived from the US Food and Drug Administration's adverse events database. All cases of CSS reported to the US Food and Drug Administration from 1997 to April 2003 were reviewed. Information about basic demographics, suspect medication use, clinical manifestations, histologic findings, ANCA staining patterns, and the presence of antibodies to myeloperoxidase (anti-MPO) or proteinase 3 (anti-PR3) was recorded when available. There were 93 case reports of CSS with sufficient documentation, including ANCA status. There were 38 (40.9%) of 93 cases with positive ANCA results, of which 15 cases reported a positive ELISA, all of which were positive for anti-MPO. ANCA negativity was associated with an increased proportion of cardiac involvement (risk difference [RD], 38.2%; 95% CI, 25.3% to 51.0%), gastrointestinal involvement (RD, 25.5%; 95% CI, 13.9% to 37.0%), pulmonary infiltrates (odds ratio, 4.9; 95% CI, 1.5-16.2), and the outcome of a life-threatening event or death (RD, 30.9%; 95% CI, 18.7% to 43.1%) when compared with anti-MPO-positive cases. ANCA negativity was associated with a decreased proportion of peripheral neuropathy (odds ratio, 0.3; 95% CI, 0.07-0.9). These findings support the hypothesis that the presence or absence of autoantibodies influences the clinical expression and severity of CSS. Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.

  20. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

    Science.gov (United States)

    Cottin, Vincent; Bel, Elisabeth; Bottero, Paolo; Dalhoff, Klaus; Humbert, Marc; Lazor, Romain; Sinico, Renato A; Sivasothy, Pasupathy; Wechsler, Michael E; Groh, Matthieu; Marchand-Adam, Sylvain; Khouatra, Chahéra; Wallaert, Benoit; Taillé, Camille; Delaval, Philippe; Cadranel, Jacques; Bonniaud, Philippe; Prévot, Grégoire; Hirschi, Sandrine; Gondouin, Anne; Dunogué, Bertrand; Chatté, Gérard; Briault, Christophe; Pagnoux, Christian; Jayne, David; Guillevin, Loïc; Cordier, Jean-François

    2017-01-01

    To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features. We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations. Copyright © 2016 Elsevier B.V. All rights reserved.

  1. Use of a Granulocyte Immunofluorescence Assay Designed for Humans for Detection of Antineutrophil Cytoplasmic Antibodies in Dogs with Chronic Enteropathies.

    Science.gov (United States)

    Florey, J; Viall, A; Streu, S; DiMuro, V; Riddle, A; Kirk, J; Perazzotti, L; Affeldt, K; Wagner, R; Vaden, S; Harris, T; Allenspach, K

    2017-07-01

    Perinuclear antineutrophil cytoplasmic antibodies (pANCA) previously have been shown to be serum markers in dogs with chronic enteropathies, with dogs that have food-responsive disease (FRD) having higher frequencies of seropositivity than dogs with steroid-responsive disease (SRD). The indirect immunofluorescence (IIF) assay used in previous publications is time-consuming to perform, with low interobserver agreement. We hypothesized that a commercially available granulocyte IIF assay designed for humans could be used to detect perinuclear antineutrophil cytoplasmic antibodies in dogs. Forty-four dogs with FRD, 20 dogs with SRD, 20 control dogs, and 38 soft-coated wheaten terrier (SCWT) or SCWT-cross dogs. A granulocyte assay designed for humans was used to detect pANCA, cANCA, and antinuclear antibodies (ANA), as well as antibodies against proteinase-3 protein (PR-3) and myeloperoxidase protein (MPO) in archived serum samples. Sensitivity of the granulocyte assay to predict FRD in dogs was 0.61 (95% confidence interval (CI), 0.45, 0.75), and specificity was 1.00 (95% CI, 0.91, 1.00). A significant association was identified between positive pANCA or cANCA result and diagnosis of FRD (P < 0.0001). Agreement between the two assays to detect ANCA in the same serum samples from SCWT with protein-losing enteropathy/protein-losing nephropathy (PLE/PLN) was substantial (kappa, 0.77; 95% CI, 0.53, 1.00). Eight ANCA-positive cases were positive for MPO or PR-3 antibodies. The granulocyte immunofluorescence assay used in our pilot study was easy and quick to perform. Agreement with the previously published method was good. Copyright © 2017 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  2. Pathogenetic and Clinical Aspects of Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitides

    Directory of Open Access Journals (Sweden)

    Peter Lamprecht

    2018-04-01

    Full Text Available Anti-neutrophil cytoplasmic autoantibodies (ANCA targeting proteinase 3 (PR3 and myeloperoxidase expressed by innate immune cells (neutrophils and monocytes are salient diagnostic and pathogenic features of small vessel vasculitis, comprising granulomatosis with polyangiitis (GPA, microscopic polyangiitis, and eosinophilic GPA. Genetic studies suggest that ANCA-associated vasculitides (AAV constitute separate diseases, which share common immunological and pathological features, but are otherwise heterogeneous. The successful therapeutic use of anti-CD20 antibodies emphasizes the prominent role of ANCA and possibly other autoantibodies in the pathogenesis of AAV. However, to elucidate causal effects in AAV, a better understanding of the complex interplay leading to the emergence of B lymphocytes that produce pathogenic ANCA remains a challenge. Different scenarios seem possible; e.g., the break of tolerance induced by a shift from non-pathogenic toward pathogenic autoantigen epitopes in inflamed tissue. This review gives a brief overview on current knowledge about genetic and epigenetic factors, barrier dysfunction and chronic non-resolving inflammation, necro-inflammatory auto-amplification of cellular death and inflammation, altered autoantigen presentation, alternative complement pathway activation, alterations within peripheral and inflamed tissue-residing T- and B-cell populations, ectopic lymphoid tissue neoformation, the characterization of PR3-specific T-cells, properties of ANCA, links between autoimmune disease and infection-triggered pathology, and animal models in AAV.

  3. Therapeutic plasma exchange in patients with pauciimmune vasculitis: Hospital Universitario San Ignacio experience; Bogotá, Colombia

    Directory of Open Access Journals (Sweden)

    Juan P. Córdoba

    2014-05-01

    Full Text Available Introduction: The vasculitis ANCAS positive, are a group of diseases with different clinical manifestations. Therapeutic plasma exchange has become an excellent tool for the treatment of these patients in specific conditions. Objective: Describe the demographic and clinical characteristics of patients and the final outcomes in patients with positive ANCAS vasculitis that required therapeutic plasma exchange (TPE. Materials and methods: All patients under 18 years old with positive ANCAS vascultiis that required TPE where included during the period of May 2010 and December 2013. Results: 13 patients where treated, with a total of 73 TPE sessions. The average age was 52,3 years (Range 17 to 70. The principal diagnosis for interventions was rapidly progressive glomerulonephritis (RPGN plus alveolar hemorrhage (63%. The average number of sessions per patient was 5,6 (range 1 a 10 with an average of plasma volume exchange per session of 1,26 (range 0.72 a 1,56. Of the 13 patients, 11 (84,6% required renal replacement therapy (RRT during hospitalization. At discharge, 36.3% recover the renal function, 27% continue on RRT and 36,3% died. There was at least one complication in 6,8% of all sessions. Conclusions: TPE is an excellent tool to treat patients with positive ANCAS vasculitis that present with RPGN, high levels of serum creatinine or dialysis need, or alveolar hemorrhage. This is a safe procedure with comparable results according to International literature.Key words: Apheresis, plasmapheresis, therapeutic plasma exchange, vasculitis, ANCA.

  4. Paraneoplastic Seronegative Pauci-Immune Glomerulonephritis Associated with Lung Adenocarcinoma Responds to Rituximab: A Case Report

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    Pragnan Kancharla

    2018-06-01

    Full Text Available Anti-neutrophil cytoplasmic antibodies (ANCA play an important role in the pathogenesis of pauci-immune renal vasculitis. However, in 10% of the cases, ANCA are absent. We present a case of a 64-year-old man with a chronic untreated hepatitis C virus infection and Middle Eastern thalassemia who was ANCA-negative when he was hospitalized due to acute kidney injury and accounts for an uncommon presentation of renal vasculitis. The patient had earlier reported to have undergone local lobectomy and adjuvant chemotherapy (carboplatin/pemetrexed for lung adenocarcinoma a month prior. IL-6 has been reported to be involved in the pathophysiological cascade causing pauci-immune glomerulonephritis amongst non-small cell lung cancer patients. Previous studies with subgroup analysis have demonstrated that ANCA negativity has been associated with more chronic glomerular lesions and less crescent formation, which tends to have a critical outcome in the renal system. However, our patient underwent kidney biopsy exhibiting active crescentic glomerulonephritis, pauci-immune type with 5 cellular crescents amongst 15 glomeruli. To our knowledge, this is the third reported case of ANCA-negative vasculitis with typical presentation on biopsy in non-small cell lung cancer patients.

  5. RUSSIAN EXPERIENCE WITH USING MONOCLONAL ANTIBODIES TO B-LYMPHOCYTES (RITUXIMAB IN SYSTEMIC VASCULITIDES ASSOCIATED WITH NEUTROPHIL CYTOPLASMIC ANTIBODIES (PRELIMINARY RESULTS OF THE RUSSIAN REGISTER NORMA

    Directory of Open Access Journals (Sweden)

    T. V. Beketova

    2014-01-01

    Full Text Available In 2013, Russia registered officially the indications for the use of monoclonal antibodies to B-lymphocytes (rituximab, RTM in systemic vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA-SV. This communication presents the preliminary results of the Russian register of the RTM application in autoimmune diseases (NORMA that has included 50 patients with ANCA-SV treated in 14 cities of the Russian Federation. Twenty-five of 50 (50% patients received repeated courses of RTM. RTM has demonstrated a high efficacy and a good profile of treatment safety in patients with ANCA-SV in real-life national clinical practice. Among 25 patients who had been followed up for over 12 months, the remission was achieved in 92% of cases, a decrease in the ANCA-SV activity was observed in 8%. The efficacy of RTM increased when performing repeated courses, while it has been noted that the positive results can be obtained by prescribing a repeated course of RTM at a reduced dose (500–1000 mg. Prescription of the repeated courses was primarily required in patients with granulomatosis and polyangiitis affecting the lungs. Care should be taken when combining RTM treatment with cytostatics (primarily with cyclophosphamide because of the risk of secondary immunodeficiency and infectious adverse events (AE, which have been the most frequent serious AE (12% in patients with ANCA-SV.

  6. Polymorphism in Self-Assembled Structures of 9-Anthracene Carboxylic Acid on Ag(111

    Directory of Open Access Journals (Sweden)

    Bo Xu

    2012-06-01

    Full Text Available Surface self-assembly process of 9-anthracene carboxylic acid (AnCA on Ag(111 was investigated using STM. Depending on the molecular surface density, four spontaneously formed and one annealed AnCA ordered phases were observed, namely a straight belt phase, a zigzag double-belt phase, two simpler dimer phases, and a kagome phase. The two high-density belt phases possess large unit cells on the scale length of 10 nm, which are seldom observed in molecular self-assembled structures. This structural diversity stems from a complicated competition of different interactions of AnCA molecules on metal surface, including intermolecular and molecular-substrate interactions, as well as the steric demand from high molecular surface density.

  7. [Antineutrophil cytoplasmic antibodies associated with infective endocarditis: Literature review].

    Science.gov (United States)

    Langlois, V; Marie, I

    2017-07-01

    Antineutrophil cytoplasmic antibodies (ANCA) associated with infective endocarditis are a rare disorder. The condition can mimic primary systemic vasculitis (i.e. granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis). Thus, a wrong diagnosis of valvular involvement related to primary systemic vasculitis can be made in patients exhibiting ANCA associated with infective endocarditis. Because treatment of both conditions is different, this wrong diagnosis will lead to dramatic consequences in these latter patients. This review reports the state of knowledge and proposes an algorithm to follow when confronted to a possible case of ANCA associated with infective endocarditis. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  8. Radiation retinopathy caused by low dose irradiation and antithyroid drug-induced systemic vasculitis

    International Nuclear Information System (INIS)

    Sonoda, Koh-hei; Ishibashi, Tatsuro

    2005-01-01

    We report on a patient with Graves' disease with radiation retinopathy caused by low-dose irradiation and antithyroid drug-induced antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. A 38-year-old woman with Graves' disease presented with bilateral blurred vision, micro-aneurysms, telangiectasia, and macular edema. The patient was examined by ophthalmoscopy and fluorescein angiography, and radiation retinopathy was diagnosed. The patient had been treated with low-dose irradiation for her Graves' ophthalmopathy a few years earlier. She also had ANCA-positive vasculitis induced by the antithyroid drug (propylthiouracil, PTU) that had been prescribed for her at that time. Because of multiple avascular areas on both retinas, she was treated by intensive retinal photocoagulation to control progressive retinopathy. The radiation doses used to treat Graves' disease ophthalmopathy are low. Nevertheless, there is still a risk of radiation retinopathy developing in patients with PTU-induced ANCA-positive vasculitis. (author)

  9. AP-VAS 2012 case report: two patients with rheumatoid arthritis suspected of relapsed microscopic polyangiitis after initiation of dialysis.

    Science.gov (United States)

    Sugahara, Mai; Nishi, Takahiro; Tanaka, Shinji; Kurita, Noriaki; Sai, Keiko; Kano, Tatsuya; Nishio, Kyosuke; Sugimoto, Tokuichiro; Mise, Naobumi

    2013-11-01

    We report two patients with rheumatoid arthritis (RA) who were suspected of microscopic polyangiitis during maintenance dialysis. Case 1 was a 52-year-old woman with RA diagnosed at the age of 38 years and treated successfully with gold compounds. At the age of 43 years, she presented with progressive renal dysfunction and abnormal urine sediments, and a renal biopsy revealed crescentic nephritis with advanced glomerular sclerosis. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was not measured on that occasion. She reached end-stage renal failure within 4 months and started peritoneal dialysis. Eight years later, soon after she was switched to hemodialysis, she developed fever of unknown origin. MPO-ANCA was elevated to 37 EU, although there were no other signs or symptoms suggestive of vasculitis. After taking prednisolone orally (10 mg/day), her fever withdrew, and MPO-ANCA became undetectable. Case 2 was a 71-year-old woman with RA diagnosed at the age of 60 years and treated with gold compounds. She developed renal failure of unknown cause (no biopsy was performed), and started hemodialysis at the age of 69 years. One year later, she presented with fever and subsequently developed cough with hemoptysis. MPO-ANCA was elevated to 62 EU. Treatment with azathioprine 50 mg and prednisolone 35 mg daily brought remarkable clinical improvement, and MPO-ANCA became undetectable. These cases highlight the importance of measuring ANCA even in RA patients on dialysis who present with fever of unknown origin or with underlying kidney disease of uncertain etiology.

  10. Gingivitis y Anticuerpos Anticitoplasmáticos de Neutrófilos en niños y adolescentes con leucemia

    OpenAIRE

    Viera, Ninoska; Rojas de Morales, Thais; Navas, Rita; Zambrano, Olga; Paz de Gudiño, Mercedes

    2004-01-01

    Introducción: Las enfermedades sistémicas pueden originar y exacerbar alteraciones en el periodonto. Se ha demostrado la presencia de Anticuerpos Anticitoplasmáticos de neutrófi los (ANCA) en pacientes con enfermedad periodontal destructiva y con enfermedades sistémicas. Objetivo: Determinar la re la ción entre gingivitis y los ANCA en niños y ado les cen tes con leucemia que asistieron al Servicio Autónomo Hospital Uni ver si ta rio de Maracaibo y a la Fundación del Hosp...

  11. Gingivitis y Anticuerpos Anticitoplasmáticos de Neutrófilos en niños y adolescentes con leucemia

    OpenAIRE

    Viera, Ninoska T.; Rojas de Morales, Thais; Navas, Rita M.; Zambrano, Olga R.; Paz de Gudiño, Mercedes

    2004-01-01

    Introducción: Las enfermedades sistémicas pueden originar y exacerbar alteraciones en el periodonto. Se ha demostrado la presencia de Anticuerpos Anticitoplasmáticos de neutrófilos (ANCA) en pacientes con enfermedad periodontal destructiva y con enfermedades sistémicas. Objetivo: Determinar la relación entre gingivitis y los ANCA en niños y adolescentes con leucemia que asistieron al Servicio Autónomo Hospital Universitario de Maracaibo y a la Fundación del Hospital de Especialidades Pediátri...

  12. Churg-Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis.

    Science.gov (United States)

    Sánchez-Vicente, J L; Gálvez-Carvajal, S; Medina-Tapia, A; Rueda, T; González-García, L; Szewc, M; Muñoz-Morales, A

    2016-11-01

    We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg-Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  13. CUTANEOUS VASCULITIDES AND CUTANEOUS MANIFESTATIONS OF SYSTEMIC VASCULITIDES

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    Vesna Jurčić

    2009-06-01

    plexes with complement activation. Typical representatives are Henoch-Schönlein purpura, cryoglobulinemic vasculitis and vasculitis in systemic lupus erythematosus. Necrotizing arteriolitis and arteritis of small arteries are usually found in ANCA positive vasculitides. Medium-sized arteries can be involved in both ANCA positive vasculitides and polyarteritis nodosa. Conclusions This article presents the clinical picture, histopathologic characteristics, pathogenetic mechanisms and treatment of some clinicopathologic entities of vasculitides, such as Henoch-Schönlein purpura, urticarial vasculitis, cryoglobulinemic vasculitis, microscopic polyangiitis, Wegener\\'s granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa.

  14. [Doctor Zamenhof (1859-1917): a physician "who hopes"!(II)].

    Science.gov (United States)

    Lellouch, Alain

    2004-01-01

    A previous paper detailed the life and the works of Louis-Lazare Zamenhof (1857-1917), a young physician, born in Lituania, of Jewish origin, specialiazed in ophtalmology. Zamenhof created a new universal language he called "Esperanto". The study continues Zamenhof's biography, from 1905, date of the first international esperantist congress, at Boulogne-sur-Mer, in France to 1917, year of Zamenhof's death, during World War I. The paper will restitute Zamenhof's ideology in the history of the ideas of the XXth century, such as nationalisms, internationalism, pacifism and universalism of Human Right.

  15. On portraits in Ravanica

    Directory of Open Access Journals (Sweden)

    Starodubcev Tatjana

    2012-01-01

    Full Text Available This research examines a badly washed-out portrait composition of Prince Lazar, Princess Milica and their sons, Stefan and Vuk, in the Ravanica church. The opinion is that it was not painted later, over the original layer of the frescoes, but that it was made simultaneously with the other wall paintings of the lowest zones of the church. Considering the age of Stefan represented in the portrait composition, it is assumed that the decoration of the church was completed around 1385. [Projekat Ministarstva nauke Republike Srbije, br. 177036: Srpska srednjovekovna umetnosti njen evropski kontekst

  16. Modeling Chronic Toxicity: A Comparison of Experimental Variability With (QSAR/Read-Across Predictions

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    Christoph Helma

    2018-04-01

    Full Text Available This study compares the accuracy of (QSAR/read-across predictions with the experimental variability of chronic lowest-observed-adverse-effect levels (LOAELs from in vivo experiments. We could demonstrate that predictions of the lazy structure-activity relationships (lazar algorithm within the applicability domain of the training data have the same variability as the experimental training data. Predictions with a lower similarity threshold (i.e., a larger distance from the applicability domain are also significantly better than random guessing, but the errors to be expected are higher and a manual inspection of prediction results is highly recommended.

  17. Thinking in a foreign language, fast and slow

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    Turula Anna

    2016-06-01

    Full Text Available Several studies (Keysar et al., 2012; Lazar et al., 2014 suggest that decisions made in a foreign language are more rational. The authors imply that when thinking in a language which is not our native tongue, analytical, slow, deep-thinking is activated. The question that underlies the present article is whether this is a characteristic of every mental operation in the foreign medium. Studies carried out by Costa et al. (2014, Geipel et al. (2015 and Hadjichristidis et al. (2015 suggest the issue is much more complex than it may seem.

  18. Reduced CD5(+) CD24(hi) CD38(hi) and interleukin-10(+) regulatory B cells in active anti-neutrophil cytoplasmic autoantibody-associated vasculitis permit increased circulating autoantibodies.

    Science.gov (United States)

    Aybar, L T; McGregor, J G; Hogan, S L; Hu, Y; Mendoza, C E; Brant, E J; Poulton, C J; Henderson, C D; Falk, R J; Bunch, D O

    2015-05-01

    Pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is B cell-dependent, although how particular B cell subsets modulate immunopathogenesis remains unknown. Although their phenotype remains controversial, regulatory B cells (Bregs ), play a role in immunological tolerance via interleukin (IL)-10. Putative CD19(+) CD24(hi) CD38(hi) and CD19(+) CD24(hi) CD27(+) Bregs were evaluated in addition to their CD5(+) subsets in 69 patients with ANCA-associated vasculitis (AAV). B cell IL-10 was verified by flow cytometry following culture with CD40 ligand and cytosine-phosphate-guanosine (CpG) DNA. Patients with active disease had decreased levels of CD5(+) CD24(hi) CD38(hi) B cells and IL-10(+) B cells compared to patients in remission and healthy controls (HCs). As IL-10(+) and CD5(+) CD24(hi) CD38(hi) B cells normalized in remission within an individual, ANCA titres decreased. The CD5(+) subset of CD24(hi) CD38(hi) B cells decreases in active disease and rebounds during remission similarly to IL-10-producing B cells. Moreover, CD5(+) B cells are enriched in the ability to produce IL-10 compared to CD5(neg) B cells. Together these results suggest that CD5 may identify functional IL-10-producing Bregs . The malfunction of Bregs during active disease due to reduced IL-10 expression may thus permit ANCA production. © 2014 British Society for Immunology.

  19. Prevalence and clinical significance of cathepsin G antibodies in systemic sclerosis

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    M. Favaro

    2011-09-01

    Full Text Available Objectives: To evaluate the prevalence and clinical significance of cathepsin G antibodies in patients affected with systemic sclerosis (SSc, scleroderma. Methods: 115 patients affected by SSc, 55 (47,8% with diffuse scleroderma (dSSc and 60 (52,2% with limited scleroderma (lSSc, were tested for cathepsin G antibodies by ELISA method. Moreover these sera were evaluated by indirect immunofluorescence (IIF on ethanol and formalin fixed human neutrophils. Results: By means of the ELISA method 16 (13,9% patients were found to be sera positive for anti-cathepsin G, 2 (12.5% of which showed a perinuclear fluorescence pattern (P-ANCA and 4 (25% an atypical ANCA staining, while 10 (62,5% were negative on IIF. The IIF on scleroderma sera revealed 5 (4,3% P-ANCA and 18 (15,7% atypical ANCA patterns. The anti-cathepsin G antibodies significantly prevailed in scleroderma sera (p=0.02 when their frequency was compared with that of healthy controls; while they were not significantly associated to any clinical or serological features of SSc patients. Conclusions: The anti-cathepsin G antibodies were significantly frequent in scleroderma sera; however, no clinical correlations were found. Thus, the significance of their presence in SSc still needs to be clarified.

  20. 18F-fluoro-deoxy-glucose positron emission tomography combined with computed tomography can reliably rule-out infection and cancer in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis suspected of disease relapse

    DEFF Research Database (Denmark)

    Frary, Evan C; Hess, Søren; Gerke, Oke

    2017-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases characterized by systemic inflammation in small- to medium-sized blood vessels. Although immunosuppressive therapy has greatly improved the prognosis for these patients, there are still...

  1. Systemic vasculitis and the gut.

    Science.gov (United States)

    Hatemi, Ibrahim; Hatemi, Gulen; Çelik, Aykut F

    2017-01-01

    Gastrointestinal system can be involved in primary and secondary vasculitides. The recent data regarding the pathophysiology, clinical findings, diagnosis, management, and outcome of gastrointestinal involvement in different types of vasculitis are reviewed. Diagnosis of gastrointestinal vasculitis may be difficult and relies mostly on imaging, because biopsy samples are hard to obtain and superficial mucosal biopsies have a low yield. There are conflicting reports on the association of antineutrophilic cytoplasmic antibodies (ANCA) type with the frequency of gastrointestinal involvement in ANCA-associated vasculitis. Pancreatitis is a rare but serious complication of ANCA-associated vasculitis. Terminal ileitis may be observed in immunoglobulin A vasculitis and can be hard to distinguish from Crohn's disease. High fecal calprotectin levels can indicate active gastrointestinal involvement in both immunoglobulin A vasculitis and Behçet's syndrome. Refractory gastrointestinal involvement in Behçet's syndrome can be treated with thalidomide and/or TNF-α antagonists. The outcome of mesenteric vasculitis in systemic lupus erythematosus can be improved with high-dose glucocorticoids and cyclophosphamide or rituximab. Gastrointestinal system can be commonly involved in immunoglobulin A vasculitis, ANCA-associated vasculitis, polyarteritis nodosa, and Behçet's syndrome and can be an important cause of morbidity and mortality. Treatment depends on the type of vasculitis and is usually with high-dose corticosteroids and immunosuppressives.

  2. Animal models of antineutrophil cytoplasm antibody-associated vasculitis.

    LENUS (Irish Health Repository)

    Salama, Alan D

    2012-01-01

    To provide an update on the experimental models that have been developed recapitulating clinical antineutrophil cytoplasm antibody (ANCA) associated vasculitis. The application of the models in the study of pathogenesis, and the therapeutic implications of this, are covered in the article by van Timmeren and Heeringa in this issue.

  3. Properdin-dependent activation and control of immune-homeostasis and autoimmunity

    NARCIS (Netherlands)

    O'Flynn, Joseph

    2014-01-01

    The complement system has been shown to have a role in various systemic autoimmune (AI) diseases which have a renal component. This includes systemic lupus erythematosus (SLE), goodpastures syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides. In particular the classical

  4. Usefulness of antineutrophil cytoplasmic autoantibodies in diagnosing and managing systemic vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    Purpose of reviewAntineutrophil cytoplasmic autoantibodies (ANCAs) are considered important diagnostic tests in the work-up of patients suspected of vasculitis. Here we discuss new developments in the methodology of testing, the pitfalls in using these tests as diagnostic tools, and the value of

  5. Recombinant protein to analyze autoantibodies to proteinase 3 in systemic vasculitis

    NARCIS (Netherlands)

    Rarok, AA; Huitema, MG; van der Leij, MJ; van der Geld, YM; Berthold, H; Schmitt, J; Stegeman, CA; Limburg, PC; Kallenberg, CGM

    2003-01-01

    The presence of antineutrophil cytoplasmic autoantibodies with specificity for proteinase 3 (PR3-ANCA) usually is detected by enzyme-linked immunosorbent assay (ELISA) with purified PR3 as a substrate. We studied the technical performance of direct and capture ELISA using a recombinant

  6. Prevention of Ovarian High-Grade Serous Carcinoma by Elucidating Its Early Changes

    Science.gov (United States)

    2014-10-01

    serous ovarian cancer carcinogenesis. Sophia HL George, Ramlogan Sowamber, Anca Milea, Noor Salman and Patricia Shaw. September 2014. Masha Rivkin Ovarian...in mesenchymal-to-epithelial transition during high-grade serous carcinogenesis. Masha Rivkin Ovarian Cancer Symposium September 2014, Seattle WA

  7. HMGB1 in vascular diseases : Its role in vascular inflammation and atherosclerosis

    NARCIS (Netherlands)

    de Souza, A. W. S.; Westra, J.; Limburg, P. C.; Bijl, M.; Kallenberg, C. G. M.

    2012-01-01

    The nuclear protein high mobility group box 1 (HMGB1) has been suggested to be involved in the pathogenesis of several vascular diseases such as systemic vasculitis and atherosclerosis. In systemic vasculitides including ANCA-associated vasculitis and Kawasaki disease, serum HMGB1 levels are higher

  8. Expression of recombinant proteinase 3, the autoantigen in Wegener's granulomatosis, in insect cells

    NARCIS (Netherlands)

    Van der Geld, YM; Smook, MLF; Huitema, MG; Harmsen, MC; Limburg, PC; Kallenberg, CGM

    2002-01-01

    Proteinase 3 (PR3) is the major autoantigen for anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis. Little is known about the major antigenic sites on PR3. To facilitate epitope mapping, PR3 was cloned in insect cells using a baculovirus expression system. Four

  9. Disease: H01468 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic... (>10% of total WBC) PR3-ANCA (+) Prednisolone [DG:DG00093] Cyclophosphamide [DG:DG00675] Rituximab [DR:D029...hy P, Guillevin L, Merkel PA, Jayne DR ... TITLE ... Rituximab for the treatment of eosinophilic granulomatosi

  10. Proceedings – Mathematical Sciences | Indian Academy of Sciences

    Indian Academy of Sciences (India)

    Author Affiliations. Radu Ioan Boţ1 Anca Dumitru2 Gert Wanka1. Faculty of Mathematics, Chemnitz University of Technology, D-09107 Chemnitz, Germany; Faculty of Mathematics and Computer Science, Babes¸-Bolyai University, Str. Kog˜alniceanu nr. 1, 400084 Cluj-Napoca, Romania ...

  11. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial

    DEFF Research Database (Denmark)

    Szpirt, Wladimir M; Heaf, James G; Petersen, Jørgen

    2011-01-01

    The use of plasma exchange (PE) for induction treatment of anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV), including Wegener's granulomatosis (WG), is still controversial. The use of PE in AAV is not commonly accepted in patients with a plasma creatinine...

  12. Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome

    NARCIS (Netherlands)

    Schuttelaar, M L A; Jonkman, M F

    We report a patient with Churg-Strauss syndrome (CSS) with asthma, eosinophilia, nasal polyposis and ANCA-associated multisystem vasculitis, who's skin eruption started with erythematous urticarial-plaques followed by haemorrhagic bullae. Histology of the plaques revealed 'flame figures' in the

  13. DETECTION OF AUTOANTIBODIES AGAINST MYELOID LYSOSOMAL-ENZYMES - A USEFUL ADJUNCT TO CLASSIFICATION OF PATIENTS WITH BIOPSY-PROVEN NECROTIZING ARTERITIS

    NARCIS (Netherlands)

    Tervaert, J.W.C.; Limburg, Piet; ELEMA, J.D.; HUITEMA, M.G.; The, T.H; Kallenberg, Cees; Horst, G.

    PURPOSE: Assessment of the value of determination of antineutrophil cytoplasmic antibodies (ANCA) and its specificities for classification of patients with biopsy-proven necrotizing arteritis. PATIENTS AND METHODS: The serum samples of 28 consecutive patients with biopsy-proven vasculitis involving

  14. A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan.

    Science.gov (United States)

    Sada, Ken-Ei; Amano, Koichi; Uehara, Ritei; Yamamura, Masahiro; Arimura, Yoshihiro; Nakamura, Yoshikazu; Makino, Hirofumi

    2014-07-01

    We conducted a cross-sectional nationwide survey to determine eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) prevalence and clinical features in Japan. Data for EGPA patients in 2008 were collected from 1,564 hospitals. In total, 965 patients were reported from 365 departments. In a second survey, clinical data for 473 patients were obtained. We estimated that 1,866 (95% CI: 1,640-2,092) patients have EGPA in Japan (prevalence, 17.8/1,000,000). Of the 473 patients in the second survey, 315 fulfilled American College of Rheumatology (ACR) criteria or Lanham's criteria for EGPA. The mean age (± SD) of the 315 at onset was 55 ± 14 years, male to female ratio 1:2. 93% of patients had neurological manifestations, which were the organ system most frequently involved. Among 277 patients tested for myeloperoxidase (MPO)-/p anti-neutrophil cytoplasmic antibody (ANCA), 139 (50%) were positive, while only 6 of 238 were positive for proteinase3 (PR3)-/cANCA. MPO-ANCA-positive patients had renal involvement, mucous membrane or ophthalmological symptoms, and ENT symptoms more frequently, whereas cutaneous lesions and cardiovascular involvement were less common. The prevalence of EGPA and the frequency of MPO-/p-ANCA-positivity in Japanese EGPA patients were mostly similar to those of Western countries. However, female predominance and a high frequency of neurological manifestations characterized Japanese patients.

  15. High Prevalence of Autoantibodies to hLAMP-2 in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Kain, Renate; Tadema, Henko; McKinney, Eoin F.; Benharkou, Alexandra; Brandes, Ricarda; Peschel, Andrea; Hubert, Virginie; Feenstra, Tjerk; Sengoelge, Guerkan; Stegeman, Coen; Heeringa, Peter; Lyons, Paul A.; Smith, Kenneth G. C.; Kallenberg, Cees; Rees, Andrew J.

    The involvement of autoantibodies to human lysosome-associated membrane protein-2 (hLAMP-2) in anti neutrophil cytoplasmic antibody (ANCA) associated vasculitis is controversial because of the absence of confirmatory data subsequent to the initial reports of their high prevalence in this disease. We

  16. The vasculitic neuropathies: an update.

    Science.gov (United States)

    Collins, Michael P

    2012-10-01

    Vasculitic neuropathy is a heterogeneous disorder that usually occurs in systemic diseases, but less commonly appears as nonsystemic vasculitic neuropathy (NSVN). This review is intended to highlight recent developments in the field of vasculitic neuropathies. A Peripheral Nerve Society guideline provides data-driven consensus recommendation on classification of vasculitic neuropathies and diagnosis/treatment of NSVN. NSVN is sometimes accompanied by subclinical inflammation of adjacent skin. Amyotrophic lateral sclerosis with sensory involvement can mimic NSVN. Systemic vasculitides with neuropathy include polyarteritis nodosa, microscopic polyangiitis (MPA), rheumatoid vasculitis, Churg-Strauss syndrome (CSS), and hepatitis C-related mixed cryoglobulinemic vasculitis (MCV). At autopsy, MPA affects limb nerves diffusely, with maximal damage in proximal/middle segments. CSS can be accompanied by antineutrophil cytoplasmic antibodies (ANCAs), but most patients with neuropathy lack ANCAs. Cryoglobulinemic neuropathies are usually caused by vasculitis, irrespective of phenotype. Two randomized trials revealed rituximab to be noninferior to cyclophosphamide for inducing remission in ANCA-associated vasculitis. Many reports also document efficacy of rituximab in MCV. Consensus guidelines on NSVN should be evaluated prospectively. MPA-associated vasculitic neuropathy results from vasculitic lesions distributed diffusely throughout peripheral extremity nerves. Rituximab is effective for ANCA-associated and cryoglobulinemic vasculitis with neuropathy.

  17. Urinary matrix metalloproteinases reflect renal damage in anti-neutrophil cytoplasm autoantibody-associated vasculitis

    NARCIS (Netherlands)

    Sanders, J.S.F.; Huitema, M.G.; Hanemaaijer, R.; Goor, H. van; Kallenberg, C.G.M.; Stegeman, C.A.

    2007-01-01

    Renal expression of MMP-2, -9, and tissue inhibitor of MMP-1 (TIMP-1) correlates with histological disease activity in anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV). We studied whether urinary and plasma levels of MMP-2, -9, and TIMP-1 reflect renal expression of these

  18. Bartonella Endocarditis and Pauci-Immune Glomerulonephritis

    Science.gov (United States)

    Raybould, Jillian E.; Raybould, Alison L.; Morales, Megan K.; Zaheer, Misbah; Lipkowitz, Michael S.; Timpone, Joseph G.; Kumar, Princy N.

    2016-01-01

    Abstract Among culture-negative endocarditis in the United States, Bartonella species are the most common cause, with Bartonella henselae and Bartonella quintana comprising the majority of cases. Kidney manifestations, particularly glomerulonephritis, are common sequelae of infectious endocarditis, with nearly half of all Bartonella patients demonstrating renal involvement. Although a pauci-immune pattern is a frequent finding in infectious endocarditis–associated glomerulonephritis, it is rarely reported in Bartonella endocarditis. Anti–neutrophil cytoplasmic antibody (ANCA) positivity can be seen with many pathogens causing endocarditis and has been previously reported with Bartonella species. In addition, ANCA-associated vasculitis can also present with renal and cardiac involvement, including noninfectious valvular vegetations and pauci-immune glomerulonephritis. Given the overlap in their clinical presentation, it is difficult to differentiate between Bartonella endocarditis and ANCA-associated vasculitis but imperative to do so to guide management decisions. We present a case of ANCA-positive Bartonella endocarditis with associated pauci-immune glomerulonephritis that was successfully treated with medical management alone. PMID:27885316

  19. Um caso de Nefrite de shunt associada a elevação transitória de anticorpos anti-dsDNA

    Directory of Open Access Journals (Sweden)

    Marta Soares

    2014-09-01

    The authors present the case of a NS with positivity for both PR3-ANCA and for anti-dsDNA in which there was no manifestation of autoimmune disease and the resolution of the infection led to the gradual decline of autoantibodies. To our knowledge this is the first report of a NS with transient elevation of anti-dsDNA.

  20. An atypical case of Wegener's granulomatosis complicated by sepsis and coxitis

    International Nuclear Information System (INIS)

    Makowski, A.; Faflik, J.

    1993-01-01

    There is presented an atypical case Wegener's granulomatosis of maxillo-nasal region without bony destruction. The case is unusual because of sepsis and purulent coxitis. The patient responded well to treatment with vincristine and cyclophosphamide. ANCA (anti-neutrophil cytoplasmic antibodies) assays have very good sensitivity and specificity for Wegener's granulomatosis. (author)

  1. RENAL ISCHEMIA-REPERFUSION INJURY CONTRIBUTES TO RENAL DAMAGE IN EXPERIMENTAL ANTI-MYELOPEROXIDASE-ASSOCIATED PROLIFERATIVE GLOMERULONEPHRITIS

    NARCIS (Netherlands)

    BROUWER, E.; Klok, P.A; HUITEMA, M.G.; Weening, J.J.; Kallenberg, Cees

    The occurrence of focal fibrinoid necrosis of capillary loops in the very early stages of ANCA-associated necrotizing crescentic glomerulonephritis (NCGN) and the increased prevalence of this disease at older age suggest that renal ischemia may play an additional role in its pathophysiology. In the

  2. Fulminant Wegener's granulomatosis: A case report

    Directory of Open Access Journals (Sweden)

    Dinić Miroslav Ž.

    2013-01-01

    Full Text Available Introduction. Granulomatosis Wegener is anti-neutrophil cytoplasmic antibodies (ANCAs-associated systemic vasculitis of unknown etiology. It is manifested as granulomatous necrotizing inflammation of the upper and lower parts of the respiratory tract, glomerulonephritis and systemic vasculitis involving most frequently the skin and oral mucous membrane. Sera markers of this disease are c-ANCA and p-ANCA. Case report. We presented a female patient aged 52 years with purpuric spots that had appeared on the lower legs ten months before admission to our hospital. The disease ran an aggressive course, and a month before admission hemorrhagic bullae, skin ulcers, hoarseness, dyspnea, generalized arthralgia, fatigue and fever had rapidly developed. Histopathological examination of a skin sample revealed necrotizing vasculitis, so that sera markers concentrations were elevated (c-ANCA, p-ANCA. There was a perforation of the nasal septum found on rhinoscopy. During hospitalization acute abdominal pain occurred, a possible tumor in the small intestine and possible granulomas in the liver were seen by multislice computed tomography (MSCT examination, with normal findings on the lungs and kidneys. The treatment started with methylprednisolone: 500 mg/d i.v. infusion for consecutive 3 days, then 60 mg/d. On exploratory laparotomy small bowel perforation and diffuse peritonitis were found. Unstable in the postoperative period, the patient died on the day 12 of hospitalization. Conclusion. The reported patient was with fulminant Wegener’s granulomatosis, dominantly with skin changes and with gastrointestinal manifestation. This case accents the need for rapid systemic clinical evaluation in a severely ill patient with unclear diagnosis.

  3. A Development of the Principle of Virtual Laws and its Conceptual Framework in Mechanics as Fundamental Relationship between Physics and Mathematics

    Directory of Open Access Journals (Sweden)

    Raffaele Pisano

    2017-06-01

    Full Text Available Generally speaking, virtual displacement or work concerns to a timely idea according to which a motion of a certain body is not the unique possible motion. The process of reducing this motion to a particular magnitude and concept, eventually minimizing as a hypothesis, can be traced back to the Aristotelian school. In the history and philosophy of science one finds various enunciations of the Principle of Virtual Laws and its virtual displacement or work applications, i.e., from Aristotle to Leibniz’s vis viva, from Maupertuis’ least action to Euler and Lagrange with calculus of variations (statics and dynamics to Lazare Carnot’s mechanics. In this case study, I will demonstrate that a particular approach used by Lazare Carnot is original by explaining within the historical context of rival approaches such as the development of the Principle of virtual Laws (also known as the Principle of virtual velocity or of virtual work. I will also discuss Carnot’s geometric motion as one of the possible but invertible movements applied to virtual displacement as employed in his theories of machines and collisions. I will then go on to explore how the originality of an invertible motion within his mechanical and, in general, mathematical research program permitted Carnot to introduce a new way of structuring a scientific theory and making mechanics, with respect to the Newtonian paradigm, to scholars and his students of the École polytechnique de Paris.

  4. Advantages and Disadvantages of Exempting Municipal Bonds from the Federal Income Tax: The U.S. Experience

    Directory of Open Access Journals (Sweden)

    Esteban G. DALEHITE

    2007-02-01

    Full Text Available Romania and other Eastern European countries have undergone dramatic reforms as they have sought to democratize political institutions, develop their economies, rely on private markets for the provision of goods and services, and pursue a course of economic integration with Western European nations (Lazar, 2005. Of course, these reforms have included the complete overhaul of tax and revenue systems (Lazar, 2005. As these tax reforms mature and are adapted to the differing realities of each country, it might be useful to reflect on the experiences and mistakes of countries whose tax systems they have used as blueprint for their own reforms. This is the spirit in which this analysis is written. The article presents a synthesis of the American experience with tax-exempt municipal bonds, and the advantages and disadvantages associated with this tax exemption. The exemption represents a subsidy from the federal government to states and local governments, and as such, it has powerful incentives with implications from the economic and redistributive standpoints. This article explains these implications and how they have been addressed in the U.S.

  5. Anti-glomerular basement membrane (anti-GBM) disease accompanied by vasculitis that was not positive for antineutrophil cytoplasmic antibodies to myeloperoxidase and proteinase 3: a report of two cases and the incidence of anti-GBM disease at one institution.

    Science.gov (United States)

    Nakabayashi, Kimimasa; Fujioka, Yasunori; Arimura, Yoshihiro; Fukuoka, Toshihito; Marumo, Tomohumi; Umino, Michiru; Kamiya, Yasushi; Okai, Takahiro; Tsurumaki, Shigeru; Nagasawa, Toshihiko; Yamada, Akira

    2011-08-01

    Anti-glomerular basement membrane (anti-GBM) disease is thought to be distinct from vasculitis. In contrast, there have been several papers suggesting the presence of angiitis in cases that were positive for anti-GBM antibody (Ab), as well as for either myeloperoxidase (MPO)- or proteinase 3 (PR3)-anti-neutrophil cytoplasmic antibody (ANCA) (Group I). We experienced four patients who had anti-GBM Abs, but not MPO- and PR3-ANCA (Group II), and two of these patients were found to have vasculitis. Therefore, we performed an in-depth study on these two patients. The patients with anti-GBM disease were isolated from 578 cases whose renal tissues were examined, and they were categorized into two groups. We have already published the data about Group I. We then proceeded to study two vasculitic patients in Group II clinically, pathologically, and serologically. The anti-GBM Ab and ANCA levels were detected by enzyme-linked immunosorbent assays. Renal specimens were studied by routine staining as well as immunohistochemical investigations of CD31 and type IV collagen. The total number of patients with anti-GBM disease was 7 (7/578 = 1.2%), with 3 patients belonging to Group I and 4 patients belonging to Group II. Two patients in Group II were diagnosed to have vasculitis, but the remaining 2 patients did not. One vasculitic patient was complicated by pulmonary hemorrhage, while the other vasculitic patient displayed peripheral neuropathy as well as a small cavity lesion in the lung. The latter patient was found to be positive for perinuclear (p)-ANCA, but not for any other ANCA subsets. The renal pathology in the two vasculitic patients showed crescentic glomerulonephritis (CSGN) and immunoglobulin (Ig) G linear deposits along the glomerular capillary loops. The former patient showed fibrinoid angiitis in an afferent arteriole as well as peritubular capillaritis. The latter patient demonstrated peritubular capillaritis. These peritubular capillaritides were diagnosed by

  6. Antibodies against linear epitopes on Goodpasture autoantigen in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.

    Science.gov (United States)

    Jia, Xiao-Yu; Yu, Jun-Tao; Hu, Shui-Yi; Li, Jian-Nan; Wang, Miao; Wang, Chen; Chen, Min; Cui, Zhao; Zhao, Ming-Hui

    2017-09-01

    In a substantial number of patients with crescentic glomerulonephritis, both anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) are detected simultaneously. ANCA is presumed to be the initial event but the mechanism is unknown. In the present study, we investigated the antibodies against linear epitopes on Goodpasture autoantigen in sera from patients with ANCA-associated vasculitis, aiming to reveal the mechanisms of the coexistence of the two kinds of autoantibodies. Thirty-one patients with ANCA-associated vasculitis were enrolled in this study. Twenty-four overlapping linear peptides were synthesized across the whole sequence of Goodpasture autoantigen. Serum antibodies against linear peptides were detected by ELISA and their associations with clinical features were further analyzed. Twenty-five out of the thirty-one (80.6%) sera from patients with ANCA-associated vasculitis possessed antibodies against linear peptides on Goodpasture autoantigen. These antibodies could be detected in 50% of patients with normal renal function (Scr ≤ 133 μmol/L), 70% of patients with moderate renal dysfunction (133 μmol/L  600 μmol/L) (P = 0.032). The highest recognition frequencies were found for peptides P4 (51.6%), P14 (54.8%), and P24 (54.8%), which contained the sequences that constitute the conformational epitopes of E A (P4) and E B (P14) recognized by anti-GBM antibodies. The level of anti-P4 antibodies was positively correlated with the percentage of crescents in glomeruli (r = 0.764, P = 0.027). Patients with anti-P24 antibodies had a significantly higher prevalence of renal dysfunction on diagnosis (88.2 vs. 42.9%, P = 0.018). Antibodies against linear epitopes on Goodpasture autoantigen could be detected in sera of patients with ANCA-associated vasculitis, which might mediate the production of antibodies towards the conformational epitopes on Goodpasture autoantigen, namely, the anti-GBM antibodies.

  7. Cocaine-induced vasculitis with cutaneous manifestation: A recurrent episode after 2 years

    Directory of Open Access Journals (Sweden)

    Thein Swe

    2016-01-01

    Full Text Available Cocaine is a popular recreational drug in the United States, and up to 70% of the seized cocaine contains levamisole which is an antihelminthic that can cause cutaneous vasculitis with necrosis and positive antineutrophil cytoplasmic antibodies (ANCAs. Here, we report a unique case of recurrent cocaine-induced vasculitis in a patient who smokes cocaine for more than 20 years. A 38-year-old woman complained of painful erythematous rash in her right arm and right thigh which appeared some hours after smoking cocaine. Physical examination revealed tender, erythematous base, retiform purpura with necrosis and bullae. Serological test showed high atypical perinuclear ANCA titer of 1:320 and antimyeloperoxidase antibody level of 20.4 U/mL. Cocaine-induced vasculitis should be one of the differential diagnoses in cocaine abusers who present with painful rash and areas of necrosis. Early diagnosis is important since it is an emerging public health concern.

  8. Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood.

    Science.gov (United States)

    Kara, Ozlem; Demirel, Fatma; Acar, Banu Celikel; Cakar, Nilgün

    2013-01-01

    Wegener granulomatosis (WG) is a cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)-associated, multi-system, necrotizing granulomatous vasculitis. Inflammation of the nasal or oral mucosa, and lung and kidney involvements are typical in the course of the disease. In rare cases, pituitary involvement may occur and cause panhypopituitarism. Pituitary involvement is very rare, and only two pediatric case reports have been published to date out of a total of 24 cases. This is a case report of an adolescent patient who presented with panhypopituitarism symptoms and was later diagnosed with WG. A 16-year-old female patient complained of fever, headache, purulent nasal discharge and severe muscle and joint pain. Additionally, she had polyuria and polydipsia. Investigations revealed a pituitary mass and panhypopituitarism. Positivity of c-ANCA and renal biopsy result compatible with WG confirmed the diagnosis.

  9. The prevalence of genetic and serologic markers in an unselected European population-based cohort of IBD patients

    DEFF Research Database (Denmark)

    Riis, Lene; Vind, Ida; Vermeire, Severine

    2007-01-01

    BACKGROUND AND AIM: The aetiology of inflammatory bowel disease (IBD) is unknown, but it has become evident that genetic factors are involved in disease susceptibility. Studies have suggested a north-south gradient in the incidence of IBD, raising the question whether this difference is caused...... by genetic heterogeneity. We aimed to investigate the prevalence of polymorphisms in CARD15 and TLR4 and occurrence of anti-Saccharomyces cerevisiae (ASCA) and antineutrophil cytoplasmic antibodies (pANCA) in a European population-based IBD cohort. METHODS: Individuals from the incident cohort were genotyped...... for three mutations in CARD15 and the Asp299gly mutation in TLR4. Levels of ASCA and pANCA were assessed. Disease location and behaviour at time of diagnosis was obtained from patient files. RESULTS: Overall CARD15 mutation rate was 23.9% for CD and 9.6% for UC patients (P

  10. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial

    DEFF Research Database (Denmark)

    de Groot, Kirsten; Harper, Lorraine; Jayne, David R W

    2009-01-01

    BACKGROUND: Current therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis are limited by toxicity. OBJECTIVE: To compare pulse cyclophosphamide with daily oral cyclophosphamide for induction of remission. DESIGN: Randomized, controlled trial. Random assignments were...... outcome); change in renal function, adverse events, and cumulative dose of cyclophosphamide (secondary outcomes). RESULTS: Groups did not differ in time to remission (hazard ratio, 1.098 [95% CI, 0.78 to 1.55]; P = 0.59) or proportion of patients who achieved remission at 9 months (88.1% vs. 87...... regimen induced remission of ANCA-associated vasculitis as well as the daily oral regimen at a reduced cumulative cyclophosphamide dose and caused fewer cases of leukopenia. PRIMARY FUNDING SOURCE: The European Union....

  11. An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

    Directory of Open Access Journals (Sweden)

    Sujit Surendran

    2017-01-01

    Full Text Available We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome. We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA and proteinase (PR-3 antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA. Our patient had ANCA-associated vasculitis (AAV with features suggestive of both GPA and EGPA. She was treated with methylprednisolone and cyclophosphamide and attained remission after 2 weeks of therapy. This is a rare report of a patient with AAV having features of both EGPA and GPA.

  12. Cutting edge issues in the Churg-Strauss syndrome.

    Science.gov (United States)

    Szczeklik, Wojciech; Jakieła, Bogdan; Adamek, Dariusz; Musiał, Jacek

    2013-02-01

    Churg-Strauss syndrome (CSS) is a rare systemic small-vessel vasculitis that develops in the background of bronchial asthma, which is characterized by eosinophilia and eosinophilic infiltration of various tissues. It belongs to the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides. The triggering factors and pathogenesis of CSS are still unknown. The possible role of eotaxin-3 and CCR4-related chemokines in selective recruitment of eosinophils to the target tissues in CSS has been recently suggested, but the role of eosinophilic inflammation in the development of vasculitic lesions is not completely understood. From the clinical view, two distinct phenotypes of the disease are slowly emerging depending on the ANCA-positivity status. Glucocorticoids are still the mainstay of treatment; however, data are accumulating regarding the beneficial role of novel immunosuppressants and biologic compounds, especially in patients with poorer prognosis.

  13. [A case of Churg-Strauss syndrome with short duration from the onset of asthma to diagnosis of vasculitis].

    Science.gov (United States)

    Fuse, Yoshikazu

    2013-01-01

    A 68-year-old woman was hospitalized because of bronchial asthma and a high myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) level. She had suffered from rhinitis from one year before hospitalization, body weight loss from three months before, and asthma from one month before. On admission, she complained of dyspnea and body weight loss of over 6 kg. On laboratory tests, high MPO-ANCA and urinary abnormalities were found. On the next day, a renal biopsy was performed and histology showed necrotizing vasculitis with cellular crescents. Churg-Strauss syndrome (CSS) was diagnosed on the basis of the clinical course and histological findings. Prednisolone therapy induced rapid symptom remission, which was achieved within one month from the onset of asthma to the diagnosis of CSS. Early diagnosis and early care led to a good prognosis.

  14. B Cell Depletion: Rituximab in Glomerular Disease and Transplantation

    Directory of Open Access Journals (Sweden)

    S. Marinaki

    2013-12-01

    Full Text Available B cells play a central role in the pathogenesis of many autoimmune diseases. Selective targeting can be achieved with the use of the monoclonal antibody rituximab. In addition to being a drug for non-Hodgkin's lymphoma, rituximab is also an FDA-approved treatment for refractory rheumatoid arthritis and, since recently, ANCA vasculitis. It has shown efficacy in many autoimmune diseases. This review will discuss current evidence and the rationale of the use of rituximab in glomerular diseases, including randomized controlled trials. The focus will be on the use of rituximab in idiopathic membranous nephropathy, systemic lupus erythematosus and ANCA-associated vasculitis. The emerging role of rituximab in renal transplantation, where it seems to be important for the desensitization protocols for highly sensitized patients as well as for the preconditioning of ABO-incompatible recipients and the treatment of antibody-mediated rejection, will also be addressed.

  15. Isolated pauci-immune pulmonary capillaritis

    Directory of Open Access Journals (Sweden)

    Ashok Kumar Mehrotra

    2015-01-01

    Full Text Available A young house wife presented with low grade fever, cough, haemoptysis and SOB of unknown aetiology for 40 days duration. Respiratory system examination revealed diffuse crepts and rhonchi. Other organ system examination did not reveal any abnormality. X-ray chest PA view and CT thorax showed diffuse bilateral necrotising nodular lesions of various sizes with small pleural effusion. She also had low resting oxygen saturation with falling haematocrit. Her Serum was week positive for p-ANCA and negative for MPO-ANCA. Bronchoscopy revealed continuous bloody aspirates. We could not isolate any organisms in any of the specimens from her and she was unresponsive to any of the antibiotics either. Based on the clinical, laboratory data, radiological features and positive outcome to pulse therapy of methylprednisolone and cyclophosphamide, she was diagnosed as a case of IPIPC.

  16. Progranulin antibodies in autoimmune diseases.

    Science.gov (United States)

    Thurner, Lorenz; Preuss, Klaus-Dieter; Fadle, Natalie; Regitz, Evi; Klemm, Philipp; Zaks, Marina; Kemele, Maria; Hasenfus, Andrea; Csernok, Elena; Gross, Wolfgang L; Pasquali, Jean-Louis; Martin, Thierry; Bohle, Rainer Maria; Pfreundschuh, Michael

    2013-05-01

    Systemic vasculitides constitute a heterogeneous group of diseases. Autoimmunity mediated by B lymphocytes and their humoral effector mechanisms play a major role in ANCA-associated vasculitis (AAV) as well as in non-ANCA associated primary systemic vasculitides and in the different types of autoimmune connective tissue disorders and rheumatoid arthritis. In order to detect autoantibodies in systemic vasculitides, we screened protein macroarrays of human cDNA expression libraries with sera from patients with ANCA-associated and ANCA-negative primary systemic vasculitides. This approach led to the identification of antibodies against progranulin, a 88 kDA secreted glycoprotein with strong anti-inflammatory activity in the course of disease of giant-cell arteritis/polymyalgia rheumatica (14/65), Takayasu's arteritis (4/13), classical panarteritis nodosa (4/10), Behcet's disease (2/6) and in the course of disease in granulomatosis with polyangiitis (31/75), Churg-Strauss syndrome (7/23) and in microscopic polyangiitis (7/19). In extended screenings the progranulin antibodies were also detected in other autoimmune diseases such as systemic lupus erythematosus (39/91) and rheumatoid arthritis (16/44). Progranulin antibodies were detected only in 1 of 97 healthy controls. Anti-progranulin positive patients with systemic vasculitides, systemic lupus erythematosus or rheumatoid arthritis had significant lower progranulin plasma levels, indicating a neutralizing effect. In light of the anti-inflammatory effects of progranulin, progranulin antibodies might exert pro-inflammatory effects thus contributing to the pathogenesis of the respective autoimmune diseases and might serve as a marker for disease activity. This hypothesis is supported by the fact that a positive progranulin antibody status was associated with active disease in granulomatosis with polyangiitis. Copyright © 2012 Elsevier Ltd. All rights reserved.

  17. AP-VAS 2012 case report: two patients with rheumatoid arthritis suspected of relapsed microscopic polyangiitis after initiation of dialysis

    OpenAIRE

    Sugahara, Mai; Nishi, Takahiro; Tanaka, Shinji; Kurita, Noriaki; Sai, Keiko; Kano, Tatsuya; Nishio, Kyosuke; Sugimoto, Tokuichiro; Mise, Naobumi

    2013-01-01

    We report two patients with rheumatoid arthritis (RA) who were suspected of microscopic polyangiitis during maintenance dialysis. Case 1 was a 52-year-old woman with RA diagnosed at the age of 38 years and treated successfully with gold compounds. At the age of 43 years, she presented with progressive renal dysfunction and abnormal urine sediments, and a renal biopsy revealed crescentic nephritis with advanced glomerular sclerosis. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA...

  18. “Peripheral Neuropathy Crippling Bronchial Asthma”: Two Rare Case Reports of Churg-Strauss Syndrome

    Directory of Open Access Journals (Sweden)

    Kamal Kishore Pandita

    2014-01-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare cause of vasculitic neuropathy. Although rare and potentially fatal, Churg-Strauss syndrome (CSS is easily diagnosable and treatable. The presence of bronchial asthma with peripheral neuropathy in a patient alerts a physician to this diagnosis. This is vividly illustrated by the presented two cases who had neuropathy associated with bronchial asthma, eosinophilia, sinusitis, and positive perinuclear antineutrophil cytoplasmic antibodies (p-ANCA test, which improved with administration of steroids.

  19. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome).

    Science.gov (United States)

    Mouthon, Luc; Dunogue, Bertrand; Guillevin, Loïc

    2014-01-01

    Recently, a group of experts in the field suggested to rename Churg-Strauss syndrome as eosinophilic granulomatosis with polyangiitis (EGPA). This condition, first described in 1951, is a rare small- and medium-sized-vessel vasculitis characterized by an almost constant association with asthma and eosinophilia, and, by the presence of anti-myeloperoxidase (MPO) antineutrophil cytoplasm antibodies (ANCA) in 30-38% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient. Asthma is severe, associated with eosinophilia and extrapulmonary symptoms. Most frequently EGPA involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Recent insights, particularly concerning clinical differences associated with ANCA status, showed that EGPA patients might constitute a heterogeneous group. Thus, EGPA patients with anti-MPO ANCA suffered more, albeit not exclusively, from vasculitis symptoms, such as glomerulonephritis, mononeuritis multiplex and alveolar hemorrhage, whereas ANCA-negative patients more frequently develop heart involvement. This observation led to the hypothesis that EGPA might be divided into different clinical and pathophysiological subtypes, which could be managed better with more specifically adapted therapies. For now, EGPA treatment still relies mainly on corticosteroids and, when necessary for patients with poorer prognoses, combined immunosuppressant drugs, especially cyclophosphamide. Overall survival of EGPA patients is good, despite not uncommon relapses. Copyright © 2014 Elsevier Ltd. All rights reserved.

  20. Concurrent IgG4-related tubulointerstitial nephritis and IgG4 myeloperoxidase-anti-neutrophil cytoplasmic antibody positive crescentic glomerulonephritis: A case report.

    Science.gov (United States)

    Su, Tao; Yang, Li; Cui, Zhao; Wang, Su-Xia; Zhao, Ming-Hui

    2017-05-01

    IgG4-related disease (IgG4-RD) is a newly recognized systemic disease. The typical pathological finding in the kidney is abundant IgG4-positive plasma cell infiltration with characteristic storiform fibrosis in the interstitium. Antibodies of the IgG4 subclass have been linked to certain autoimmune diseases including antiproteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) of the IgG4 subclass. Here, we report a rare case of kidney injury with concurrent typical IgG4-related tubulointerstitial nephritis and IgG4 subclass of myeloperoxidase (MPO) ANCA-positive necrotizing crescentic glomerulonephritis. A 42-year-old Chinese man presented with repeated epigastric pain, sausage-shaped pancreas observed morphologically in computed tomography, effectiveness of prednisone therapy and was diagnosed with autoimmune pancreatitis. He subsequently developed acute kidney injury. The patient had an elevated serum IgG4, eosinophilia, and positive MPO-ANCA of IgG4-dominant subclass. Renal biopsy revealed necrotizing crescentic nephritis and typical IgG4-related tubulointerstitial nephritis. The patient was treated with a combination of corticosteroids and cyclophosphamide, and a course of rituximab was later added to deplete peripheral B cells. The patient responded well and his renal function improved. This is the first case report of an IgG4-RD with concurrent IgG4-related tubulointerstitial nephritis and IgG4 MPO-ANCA-associated necrotizing crescentic glomerulonephritis. It raises the difficulty in differentiation diagnosis of the two separate diseases that is worthy of further study.

  1. Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos Pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibody-positive vasculitis

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    Marcelo Fernández Casares

    2012-08-01

    Full Text Available Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA positivos (VAA, son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP, sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.The most frequently observed pulmonary complications of vasculitis (AAV with anti-neutrophil cytoplasmic positive antibodies (ANCA are alveolar hemorrhage, granulomas and airway stenosis. In recent years, some reports have been published that show the association of vasculitis with pulmonary fibrosis (PF, suggesting that it may be another complication of AAV. We report and describe here two cases with such association, and their clinical, tomographic and immunological characteristics. Given that in the association between PF and AAV, as reported in the last years, PF could be the first manifestation of AAV, the search for ANCA in patients with PF may be necessary, as a cause of it and for the possible subsequent development of vasculitis.

  2. Microscopic polyangiitis: Atypical presentation with extensive small bowel necrosis, diffuse alveolar hemorrhage, and renal failure

    OpenAIRE

    Segraves, Justin M.; Iyer, Vivek N.

    2017-01-01

    Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside ...

  3. Circumvention of normal constraints on granule protein gene expression in peripheral blood neutrophils and monocytes of patients with antineutrophil cytoplasmic autoantibody-associated glomerulonephritis.

    Science.gov (United States)

    Yang, Jia Jin; Pendergraft, William F; Alcorta, David A; Nachman, Patrick H; Hogan, Susan L; Thomas, Robin P; Sullivan, Pamela; Jennette, J Charles; Falk, Ronald J; Preston, Gloria A

    2004-08-01

    Granulopoiesis-related genes are distinctively upregulated in peripheral leukocytes of patients with antineutrophil cytoplasmic autoantibodies (ANCA)-associated glomerulonephritis. Affymetrix microarrays identified the upregulation of nine neutrophilic primary granule genes, including myeloperoxidase (MPO) and proteinase 3 (PR3), plus five secondary granule genes. Coordinate expression of granulocyte maturation marker CD35, measured by TaqMan PCR, and positive in situ staining for PR3 transcripts in polymorphic neutrophils and monocytes indicate that these genes are expressed in "mature" cells. Increased transcripts correlated with disease activity and absolute neutrophil values but not with "left shift," drug regimen, cytokine levels, hematuria, proteinuria, ANCA titer, serum creatinine, gender, or age. Upregulation of PR3 and MPO transcripts was specifically associated with ANCA disease (n = 56) as these changes were not detected in patients with ESRD (n = 25) or systemic lupus erythematosus (n = 17), as determined by TaqMan PCR. This is the first report of this phenomenon in nonneoplastic cells. The data raise the hypothesis that, in addition to the presence of anti-MPO or anti-PR3 autoantibodies, a second critical component in the cause of this disease is the reactivation of once-silenced genes leading to increased antigen availability.

  4. Clinical features and prognostic factors of Churg-Strauss syndrome.

    Science.gov (United States)

    Kim, Mi-Yeong; Sohn, Kyoung-Hee; Song, Woo-Jung; Park, Heung-Woo; Cho, Sang-Heon; Min, Kyung-Up; Kang, Hye-Ryun

    2014-01-01

    Churg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans. Medical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011. Data from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not. ANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.

  5. Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis

    Directory of Open Access Journals (Sweden)

    Ana Ludueña

    2011-08-01

    Full Text Available El compromiso meníngeo es una manifestación infrecuente de la granulomatosis de Wegener. Puede manifestarse como cefalea con hiperproteinorraquia y engrosamiento de la duramadre con aspecto granulomatoso, que se observa en la resonancia magnética. Presentamos un varón de 57 años con granulomatosis de Wegener que debutó con compromiso de vías aéreas superiores, oídos, órbitas y meningitis granulomatosa asintomática y que posteriormente evolucionó con mononeuritis múltiple y glomerulonefritis crescéntica ANCA positiva. La presencia de ANCA y el compromiso sistémico (vías aéreas superiores, oído, órbitas, nervios periféricos, duramadre y glomerulonefritis rápidamente progresiva permitieron en este caso llegar a un diagnóstico de certeza e iniciar el tratamiento inmunosupresor combinado (corticoides y ciclofosfamida. Evolucionó con remisión clínica y serológica (negativización de ANCA, pero persistiendo leve deterioro secuelar auditivo y de la función renal, sin recidiva de la enfermedad de base.

  6. Belmont House Nursing Home, Galloping Green, Stillorgan, Co. Dublin.

    LENUS (Irish Health Repository)

    O'Brien, Eóin C

    2015-01-01

    ANCA vasculitis encompasses several autoimmune conditions characterised by destruction of small vessels, inflammation of the respiratory tract and glomerulonephritis. Most patients harbour autoantibodies to myeloperoxidase (MPO) or proteinase 3 (PR3). Clinical and experimental data suggest that pathogenesis is driven by ANCA-mediated activation of neutrophils and monocytes. We investigated a potential role for distinct monocyte subsets. We found that the relative proportion of intermediate monocytes is increased in patients versus control individuals, and both MPO and PR3 are preferentially expressed on these cells. We demonstrate that MPO and PR3 are expressed independently of each other on monocytes and that PR3 is not associated with CD177. MPO expression correlates with that of Fc receptor CD16 on intermediate monocytes. Monocyte subsets respond differently to antibodies directed against MPO and PR3, with anti-MPO but not anti-PR3 leading to increased IL-1β, IL-6 and IL-8 production. In concordance with the observed higher surface expression of MPO on intermediate monocytes, this subset produces the highest quantity of IL-1β in response to anti-MPO stimulation. These data suggest that monocytes, specifically, the intermediate subset, may play a role in ANCA vasculitis, and also indicate that substantial differences exist between the effect of anti-MPO and anti-PR3 antibodies on these cells.

  7. High Prevalence of Neutrophil Cytoplasmic Autoantibodies in Infants with Food Protein-Induced Proctitis/Proctocolitis: Autoimmunity Involvement?

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    Alena Sekerkova

    2015-01-01

    Full Text Available Background. Food protein-induced proctitis/proctocolitis (FPIP is the most common noninfectious colitis in children in the first year of life. Along with the overall clinical symptoms, diarrhoea and rectal bleeding are the main manifestations of the disease. There is no routine noninvasive test that would be specific for this type of colitis. The aim of our study was to find a noninvasive laboratory test or tests that may be helpful in differential diagnosis of food protein-induced proctitis/proctocolitis. Methods. ANA, ANCA, ASCA, a-EMA, a-tTg, specific IgE, total IgE, IgG, IgA, IgM, and concentration of serum calprotectin were measured in a group of 25 patients with colitis and 18 children with other diagnoses. Results. Atypical-pANCA antibodies of IgG isotype were detected in the sera of 24 patients by the method of indirect immunofluorescence, and 5 patients showed also the positivity of IgA isotype. In control samples these autoantibodies were not detected. Other autoantibodies were not demonstrated in either patient or control group. Conclusions. Of the parameters tested in noninfectious colitis, atypical-pANCA on ethanol-fixed granulocytes appears to be a suitable serological marker of food protein-induced proctitis/proctocolitis and suggests a possible involvement of an autoimmune mechanisms in the pathogenesis of this disease.

  8. Serologic Investigations in Children with Inflammatory Bowel Disease and Food Allergy

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    Urszula Grzybowska-Chlebowczyk

    2009-01-01

    Patients and methods. The study comprised 95 children at the ages of 2 to 18 years. The diagnosis of IBD was established on the basis of Porto criteria. Tests of blood serum were performed in all children: IgA and IgG ASCA, p-ANCA, c-ANCA using ELISA method. Results. IgE-dependent FA was found in 32.5% children with UC and in 21% with CD. We did not observe any relation between the occurrence of FA and the frequency and ASCA titre. p-ANCA were significantly more frequent in the group of children with UC. The occurrence of ASCA antibodies was observed in 73.7% of children with CD, 17.5% with UC and almost 30% with allergic colitis. Conclusions. Patients with CD and the presence of ASCA revealed a significantly more frequent localization of lesions within the small bowel and a tendency towards older age. We observed a connection between the occurrence of antibodies and the examined mutations of gene NOD2/CARD15.

  9. The memorial of Jelena Lazarević in Herzegovina

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    Pekić Radmilo B.

    2014-01-01

    Full Text Available This paper presents a medieval church which was found by accident and unearthed in Cernica in Gacko. It was unknown to historiography and the general public. Namely, its construction and existence was saved via tradition, which in this case proved to be correct. According to the tradition, the church endower was Jelena, daughter of Lazar Hrebljanović, who married Sandalj Hranic, the Duke of Hum and Bosnia. It was her second marriage. A few medieval monuments - stećci were found in front of the unearthed church. On one of them, there was an inscription saying that Nikola Rasković, the Drobnjak prince, was burried there. The unearthed church and the inscription on the stećak are testimonies which confirm findings of the former explorations concerning the century-old existence of the Serbs and Orthodox belief not only on the area of Cernica but also on the area of the medieval Hum.

  10. Autun et Perrecy-les-Forges (Saône-et-Loire

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    Sylvie Balcon-Berry

    2003-10-01

    Full Text Available AutunÀ Autun, deux opérations ont été menées en parallèle. Il s’agit tout d’abord de l’étude archéologique de l’ancienne "salle capitulaire" du cloître canonial de Saint-Nazaire, puis des relevés du porche de la cathédrale Saint-Lazare.Étude archéologique de Saint-NazaireEn 2002, s’est poursuivie l’analyse de l’enfoui et des élévations de l’ancienne "salle capitulaire" située à l’est de la galerie orientale. Il a notamment été possible de dégager plus amplement les parties inférieures du mur...

  11. Uus sünagoog pani juudid tantsima / Peeter Kuimet, Kaia Otti

    Index Scriptorium Estoniae

    Kuimet, Peeter

    2007-01-01

    Tallinnas Karu tänaval avati 16. mail 2007 uus juudi sünagoog, avamistseremoonial viibisid Iisraeli asepeaminister Shimon Peres, Eesti president Toomas Hendrik Ilves, peaminister Andrus Ansip, Venemaa pearabi Berl Lazar, Iisraeli pearabi Yona Metzger ja sünagoogi suurrahastaja Aleksander Bronshtein. President Toomas Hendrik Ilves viitas oma kõnes paralleelidele eestlaste ja juutide ajaloos. Vt. samas: Juutide usk, kombed ja toit; Sünagoogi avamist kommenteerivad meediaõppejõud Hagi Shein, dirigent Eri Klas. Vt. samas: Peres: elevantide keskel on väikestel raske magada. Ilmunud ka: Postimees : na russkom jazõke 17. mai 2007, lk. 2; Narvskaja Gazeta, 19. mai 2007, lk. 6 (autor Ksenia Repson)

  12. Severe maxillary osteomyelitis in a Gray Wolf (Canis lupus)

    Science.gov (United States)

    Barber-Meyer, Shannon

    2012-01-01

    Dental injuries to or abnormalities in functionally important teeth and associated bones in predators may significantly reduce the ability to kill and consume prey (Lazar et al. 2009). This impairment is likely exacerbated in coursing predators, such as Gray Wolves, that bite and hold onto fleeing and kicking prey with their teeth. Damage to carnassials (upper fourth premolar, P4, and lower first molar, M1) and associated bones in Gray Wolves may especially inhibit the consumption of prey because these teeth slice meat and crush bone. Here I report maxillary osteomyelitis involving the carnassials in a wild Gray Wolf from northeastern Minnesota of such severity that I hypothesize it ultimately caused the Gray Wolf to starve to death.

  13. Cómo hacer necropolíticas en casa: Ideología de género y acuerdos de paz en Colombia

    OpenAIRE

    Muelle, Camila Esguerra

    2017-01-01

    Resumen Este artículo propone el análisis de un acervo de discursos audiovisuales y escritos difundidos por sectores que fomentaron la votación por el “no” en el plebiscito por la paz, realizado el 2 de octubre de 2016 en Colombia, así como de discursos que produjeron agentes institucionales en el marco del debate en torno a la llamada “ideología de género”. Seguiré las propuestas metodológicas de Van Dijk, Lazar y de Kress y Van Leeuwen para lograr una aproximación desde el análisis crítico ...

  14. Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis Granulomatous meningitis, crescentic glomerulonephritis and vasculitis

    Directory of Open Access Journals (Sweden)

    Ana Ludueña

    2011-08-01

    Full Text Available El compromiso meníngeo es una manifestación infrecuente de la granulomatosis de Wegener. Puede manifestarse como cefalea con hiperproteinorraquia y engrosamiento de la duramadre con aspecto granulomatoso, que se observa en la resonancia magnética. Presentamos un varón de 57 años con granulomatosis de Wegener que debutó con compromiso de vías aéreas superiores, oídos, órbitas y meningitis granulomatosa asintomática y que posteriormente evolucionó con mononeuritis múltiple y glomerulonefritis crescéntica ANCA positiva. La presencia de ANCA y el compromiso sistémico (vías aéreas superiores, oído, órbitas, nervios periféricos, duramadre y glomerulonefritis rápidamente progresiva permitieron en este caso llegar a un diagnóstico de certeza e iniciar el tratamiento inmunosupresor combinado (corticoides y ciclofosfamida. Evolucionó con remisión clínica y serológica (negativización de ANCA, pero persistiendo leve deterioro secuelar auditivo y de la función renal, sin recidiva de la enfermedad de base.Meningeal involvement is an infrequent manifestation of Wegener's granulomatosis. Clinical manifestations can be headache with high protein level in the cerebrospinal fluid and an enhanced MRI signal of granulomatous thickening of the duramater in the brain. We report a 57 year-old male with Wegener granulomatosis with onset manifestations of asymptomatic granulomatous meningitis, upper respiratory tract, ears and orbits involvement. He progressively developed ANCA positive multiple mononeuritis and crescentic glomerulonephritis. The diagnostic confirmation of Wegener's granulomatosis based on a positive ANCA test and on the evidence of systemic disease (crescentic glomerulonephritis and involvement of the upper respiratory tract, ears, orbits, peripheral nerves and duramater allowed a prompt initiation of aggressive immunosuppressive treatment with systemic cyclophosphamide and high - dosis corticosteroids. The patient entered

  15. Romanian Association of Balneology Conference – 2015, 28 – 31 May, Băile Tuşnad

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    MUNTEANU Constantin

    2015-05-01

    PERIODONTITIS TREATMENT 17.\tDr. Tatar Daniela Monica, Dr. Dogaru Gabriela, Dr. Ungur Rodica - INFLUENCE OF NATURAL FACTORS ON BRONCHIAL ASTHMA 18.\tIspas Alexandra, Gabriela Dogaru, Motricala Marieta - EFFECTS OF NATURAL THERAPEUTIC FACTORS IN BĂILE TUSNAD ON THE REHABILITATION OF POST-STROKE PATIENTS 19.\tMaria Daniela Crăciun - IMPROVING THE QUALITY OF LIFE IN CLIMAX WITH KINETOTHERAPY AND NATURAL FACTORS IN VATRA DORNEI RESORT 20.\tDenisa Muresan, Gabriela Dogaru, Motricala Marieta - EFFECTIVENESS OF NATURAL THERAPEUTIC FACTORS IN BĂILE TUSNAD FOR THE REHABILITATION OF PATIENTS WITH PARKINSON’S DISEASE 21.\tGabriel Ranghiuc, Roxana Hodorogea, Asist.Dr.BioIng. Cătălina LUCA - STUDIUL TULBURĂRILOR MUSCULO-SCHELETALE UTILIZÂND ELECTROGONIOMETRIA ŞI ELECTROMIOGRAFIA 22.\tMonica Delia POP, Letitia Mihaela Morar - How do we protect ourselves of MALPRACTICE? MEDICAL MALPRACTICE - ACTUALITY, PERSPECTIVES AND SIGNIFICANTLY JURISPRUDENCE 23.\tOctavian D Olariu, Claudia Dascal, Ionut Cadar - REHABILITATION “KEY POINTS” IN ZONE V FLEXOR TENDONS INJURIES 24.\tSuceveanu Mihaela, Pop Dana, Suceveanu Paul, Sitar Tǎut Adela Viviana, Zdrenghea Dumitru, Hâncu Nicolae - EFFECTS OF CARDIOVASCULAR REHABILITATION IN PATIENTS ADMITTED TO THE “Dr Benedek Geza” Hospital of Rehabilitation IN CARDIOVASCULAR Diseases, COVASNA 25.\tCadar D Ionut, Dogaru B Gabriela - THE PHYSICAL THERAPY ROLE IN FUNCTIONAL REHABILITATION AFTER TOTAL SHOULDER ARTHROPLASTY 26.\tDr. Glogojeanu Remus Relu, Dr. Bucur Ileana, Dr. Dogaru Gabriela, Kt. Glogojeanu Olivia Daniela - THE SIGNIFICANT RISKS FOR THE DEVELOPMENT OF OSTEOPOROSIS IN THE AERONAUTICAL PERSONNEL. PREVENTION AND TREATMENT 27.\tTatiana Bihari, Denes Marton, Doina Moldovan - SILICONE IMPLANT ARTHROPLASTY OF THE PROXIMAL INTERPHALANGEAL JOINT (PIPJ OF FINGER V OF THE RIGHT HAND – A THERAPEUTIC VARIANT IN POSTTRAUMATIC ARTHROSIS 28.\tPop Daniela Dogaru Gabriela, Stanescu Ioana, Pop Ioana - ROLE OF MEDICAL REHABILITATION TREATMENT IN POST

  16. Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.

    Science.gov (United States)

    Sada, Ken-ei; Yamamura, Masahiro; Harigai, Masayoshi; Fujii, Takao; Dobashi, Hiroaki; Takasaki, Yoshinari; Ito, Satoshi; Yamada, Hidehiro; Wada, Takashi; Hirahashi, Junichi; Arimura, Yoshihiro; Makino, Hirofumi

    2014-04-23

    We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study. In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated. Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95). MPO-ANCA

  17. Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking.

    Science.gov (United States)

    Gutierrez, Paulo Sampaio; Aiello, Vera Demarchi

    2014-01-01

    Microscopic polyangiitis (MPA) is part of the anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis, which usually presents as renal pulmonary syndrome. It is defined as a pauci-immune necrotizing small vessel vasculitis, which usually affects the kidneys, followed by the lungs. It also presents systemic symptoms. The etiology of MPA is still unclear, but evidence reinforces the autoimmune mechanisms as the main etiopathogenic factor. Aortic valve stenosis (AS) is not an uncommon disease whose etiology varies according to geographical differences and the patient's age. The natural history of AS begins with a prolonged asymptomatic period, but when symptomatic, respiratory failure is one of its main clinical presentations. The authors present the case of a 55-year-old woman who was admitted with the diagnosis of renal failure, anemia, and a cardiac murmur. The patient had been recently diagnosed with pneumonia. During hospitalization, diagnostic workup disclosed a normal kidney size as well as parenchymal thickness. A renal biopsy was undertaken but the specimen was exiguous, showing 4 sclerotic glomeruli and 1 glomerulus with crescentic glomerulonephritis. The search for ANCA was positive. The investigation of the cardiac murmur disclosed AS. The patient, on hemodialysis, presented episodes of respiratory failure, which was interpreted as acute pulmonary edema, but a suspicion of ANCA-related pulmonary renal syndrome was raised. However, the aortic valve replacement was prioritized. While awaiting cardiac surgery, the patient died because of respiratory insufficiency. Autopsy findings concluded that MPA with pulmonary hemorrhage due to vasculitis was the immediate cause of death. Although AS was present at autopsy and classified as moderate/severe, this lesion was a bystander in the process of this patient's end of life, demonstrating the value of autopsy for medical learning and reasoning purposes.

  18. Aortic stenosis concomitant with microscopic polyangiitis: a challenge in medical reasoning and thinking

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    Paulo Sampaio Gutierrez

    2014-03-01

    Full Text Available Microscopic polyangiitis (MPA is part of the anti-neutrophil cytoplasmic antibodies (ANCA-related vasculitis, which usually presents as renal pulmonary syndrome. It is defined as a pauci-immune necrotizing small vessel vasculitis, which usually affects the kidneys, followed by the lungs. It also presents systemic symptoms. The etiology of MPA is still unclear, but evidence reinforces the autoimmune mechanisms as the main etiopathogenic factor. Aortic valve stenosis (AS is not an uncommon disease whose etiology varies according to geographical differences and the patient’s age. The natural history of AS begins with a prolonged asymptomatic period, but when symptomatic, respiratory failure is one of its main clinical presentations. The authors present the case of a 55-year-old woman who was admitted with the diagnosis of renal failure, anemia, and a cardiac murmur. The patient had been recently diagnosed with pneumonia. During hospitalization, diagnostic workup disclosed a normal kidney size as well as parenchymal thickness. A renal biopsy was undertaken but the specimen was exiguous, showing 4 sclerotic glomeruli and 1 glomerulus with crescentic glomerulonephritis. The search for ANCA was positive. The investigation of the cardiac murmur disclosed AS. The patient, on hemodialysis, presented episodes of respiratory failure, which was interpreted as acute pulmonary edema, but a suspicion of ANCA-related pulmonary renal syndrome was raised. However, the aortic valve replacement was prioritized. While awaiting cardiac surgery, the patient died because of respiratory insufficiency. Autopsy findings concluded that MPA with pulmonary hemorrhage due to vasculitis was the immediate cause of death. Although AS was present at autopsy and classified as moderate/severe, this lesion was a bystander in the process of this patient’s end of life, demonstrating the value of autopsy for medical learning and reasoning purposes.

  19. Low serum myeloperoxidase in autistic children with gastrointestinal disease

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    Anthony J Russo

    2009-08-01

    Full Text Available Anthony J Russo1, Arthur Krigsman2, Bryan Jepson2, Andy Wakefield21Research Director, Health Research Institute/Pfeiffer Treatment Center, Warrenville, IL, USA; 2Thoughtful House Center for Children, Austin, TX, USAAim: To assess serum myeloperoxidase (MPO levels in autistic children with severe gastrointestinal (GI disease and to test the hypothesis that there is an association between serum MPO concentration and inflammatory GI disease, including antineutrophil cytoplasmic antibodies (ANCA, previously seen in a subgroup of autistic children.Subjects and methods: Serum from 40 autistic children with chronic digestive disease (most with ileo-colonic lymphoid nodular hyperplasia (LNH and inflammation of the colorectum, small bowel and/or stomach, and 48 controls (12 age-matched autistic children with no GI disease, 20 age-matched children without autism or GI disease, and 16 nonautistic individuals with no family history of autism were tested using enzyme-linked immunosorbent assays designed to quantitate serum MPO levels. MPO serum concentration of autistic children with GI disease was compared to GI disease severity (including LNH and erythema and presence of ANCA.Results: We found that a significant number of autistic children with chronic digestive disease had low serum levels of MPO. However, there was no significant relationship between these levels and severity of GI disease, including the presence of ANCA.Discussion: These results suggest a relationship between low MPO levels and GI disease seen in a subpopulation of autism spectrum disorders individuals. MPO concentration may therefore be a useful biomarker for GI disease in this group of autistic children.Keywords: autism spectrum disorders, autism, myeloperoxidase, GI disease, oxidative stress

  20. Proteinase 3 on apoptotic cells disrupts immune silencing in autoimmune vasculitis

    Science.gov (United States)

    Millet, Arnaud; Martin, Katherine R.; Bonnefoy, Francis; Saas, Philippe; Mocek, Julie; Alkan, Manal; Terrier, Benjamin; Kerstein, Anja; Tamassia, Nicola; Satyanarayanan, Senthil Kumaran; Ariel, Amiram; Ribeil, Jean-Antoine; Guillevin, Loïc; Cassatella, Marco A.; Mueller, Antje; Thieblemont, Nathalie; Lamprecht, Peter; Mouthon, Luc; Perruche, Sylvain; Witko-Sarsat, Véronique

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis that is associated with granulomatous inflammation and the presence of anti-neutrophil cytoplasmic antibodies (ANCAs) directed against proteinase 3 (PR3). We previously determined that PR3 on the surface of apoptotic neutrophils interferes with induction of antiinflammatory mechanisms following phagocytosis of these cells by macrophages. Here, we demonstrate that enzymatically active membrane-associated PR3 on apoptotic cells triggered secretion of inflammatory cytokines, including granulocyte CSF (G-CSF) and chemokines. This response required the IL-1R1/MyD88 signaling pathway and was dependent on the synthesis of NO, as macrophages from animals lacking these pathways did not exhibit a PR3-associated proinflammatory response. The PR3-induced microenvironment facilitated recruitment of inflammatory cells, such as macrophages, plasmacytoid DCs (pDCs), and neutrophils, which were observed in close proximity within granulomatous lesions in the lungs of GPA patients. In different murine models of apoptotic cell injection, the PR3-induced microenvironment instructed pDC-driven Th9/Th2 cell generation. Concomitant injection of anti-PR3 ANCAs with PR3-expressing apoptotic cells induced a Th17 response, revealing a GPA-specific mechanism of immune polarization. Accordingly, circulating CD4+ T cells from GPA patients had a skewed distribution of Th9/Th2/Th17. These results reveal that PR3 disrupts immune silencing associated with clearance of apoptotic neutrophils and provide insight into how PR3 and PR3-targeting ANCAs promote GPA pathophysiology. PMID:26436651

  1. Vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos: Clínica y tratamiento

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    María Virginia Paolini

    2013-04-01

    Full Text Available Las vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos (ANCA comprenden a un grupo de enfermedades caracterizadas por la inflamación de la pared de pequeños vasos. Analizamos las características epidemiológicas y clínicas en una serie de 47 pacientes: 23 (49% granulomatosis de Wegener (GW, 15 (32% poliangeítis microscópica (PAM y nueve (19% vasculitis limitada al riñón (VLR. La edad media al inicio de los síntomas fue de 50.7 ± 14.9 años. La manifestación clínica más frecuente fue el compromiso renal en 41 (87% pacientes, seguido por el pulmonar en 26 (55% y el otorrinolaringológico en 17 (36%. En 26 (55% se asoció compromiso renal y pulmonar. La forma clínica más frecuente fue la generalizada en 23 (49%, seguida por la grave en 18 (38%. El 89% presentaron determinaciones de ANCA positivas. Cuatro (8% no recibieron tratamiento inmunosupresor de inicio. De los 43 que recibieron tratamiento de inicio, 29 (67% tuvieron remisión completa, con un tiempo de remisión promedio de 35.3 meses. Once (26% presentaron recaídas, diez (91% recaídas mayores y uno (9% menor. Doce (28% fallecieron, siete en forma temprana y cinco durante la evolución de la enfermedad. Quince (31% evolucionaron a insuficiencia renal crónica. Los 26 pacientes en seguimiento tuvieron respuesta al tratamiento y 20 (77% de ellos estaban en remisión al finalizar el estudio. Las vasculitis asociadas a ANCA continúan siendo enfermedades de alta morbilidad y mortalidad, a pesar de las mejorías logradas con los tratamientos inmunosupresores.

  2. Clinical and laboratory characteristics of 19 patients with Churg-Strauss syndrome from a single South Australian centre.

    Science.gov (United States)

    Whyte, A F; Smith, W B; Sinkar, S N; Kette, F E; Hissaria, P

    2013-07-01

    Churg-Strauss syndrome (CSS) is a rare, idiopathic systemic vasculitis. There is emerging evidence of an association between the presence or absence of antineutrophil cytoplasmic antibodies (ANCA) and clinical phenotype. Thromboembolism is an increasingly recognised complication of the disease. Given the paucity of Australian data, the aim of this study was to examine the clinical and laboratory features of CSS in a single Australian centre. We performed a retrospective review of all patients who fulfilled the American College of Rheumatology classification criteria for CSS managed at the Department of Immunology, Royal Adelaide Hospital between 2002 and 2008. Nineteen patients were included. All patients had asthma and most had upper airway involvement. Peripheral nerve, musculoskeletal, gastrointestinal and cutaneous involvement was common. Renal and cardiac involvement was uncommon in this series. Histological confirmation was obtained in 15 patients (78.9%). Ten patients (52.6%) were ANCA+, and these were more likely to have musculoskeletal involvement, such as arthralgia or myalgia (odds ratio 57, P = 0.005). Thrombosis was a feature at diagnosis in six patients (31.6%); two of these recurred with relapse. Sixteen patients (84.2%) were followed up; five died, and mean survival was 8.9 years. This is the first Australian study to focus on CSS. Our results demonstrate similar presentation and prognosis of CSS to previous descriptions; however, we noted that musculoskeletal involvement was more common in ANCA+ patients. In our series, thrombosis was a significant complication and we suggest that thromboprophylaxis may be warranted. © 2013 The Authors; Internal Medicine Journal © 2013 Royal Australasian College of Physicians.

  3. Serum 25-hydroxyvitamin D levels in patients with Granulomatosis with Polyangiitis: association with respiratory infection

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    Mariana O. Perez

    Full Text Available OBJECTIVES: To determine the possible association of serum 25-hydroxyvitamin D (25OHD levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5 were evaluated in summer/autumn (n=27. The 25OHD levels were measured by radioimmunoassay. Disease activity was assessed by the Birmingham Vasculitis Activity Score Modified for Wegener’s Granulomatosis (BVAS/WG and antineutrophil cytoplasmic antibody (ANCA positivity. Respiratory infection was defined according the Centers for Disease Control and Prevention criteria. RESULTS: 25OHD levels were lower among patients in winter/spring than in summer/autumn (32.31±13.10 vs. 38.98±10.97 ng/mL, p=0.04. Seven patients met the criteria for respiratory infection: 5 in winter/spring and 2 in summer/autumn. Patients with respiratory infection presented lower 25OHD levels than those without infection (25.15±11.70 vs. 36.73±12.08 ng/mL, p=0.02. A higher frequency of low vitamin D levels (25OHD<20 ng/mL was observed in patients with respiratory infection (37.5% vs. 7.8, p=0.04. Serum 25OHD levels were comparable between patients with (BVAS/WG≥1 plus positive ANCA and without disease activity (BVAS/WG=0 plus negative ANCA (35.40±11.48 vs. 35.34±13.13 ng/mL, p=0.98. CONCLUSIONS: Lower 25OHD levels were associated with respiratory infection but not disease activity in granulomatosis with polyangiitis patients. Our data suggest that hypovitaminosis D could be an important risk factor for respiratory infection in granulomatosis with polyangiitis patients.

  4. Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

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    Shahedi K

    2013-10-01

    Full Text Available Kamyar Shahedi,1,2 Ramy Magdy Hanna,1,2 Oleg Melamed,1,2 James Wilson2,31Department of Medicine Olive-View UCLA Medical Center, Sylmar, CA, 2David Geffen School of Medicine at UCLA, Los Angeles, CA, 3UCLA Medical Center-UCLA Stone Center, Los Angeles, CA, USAAbstract: Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease.Keywords: ANCA-associated vasculitis, Wegener’s syndrome, pauci-immune glomerulonephritis, Salmonella arizonae, inflammatory bowel disease

  5. Systemic Lupus Erythematosus and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome in Patients With Biopsy-Proven Glomerulonephritis

    Science.gov (United States)

    Jarrot, Pierre-Andre; Chiche, Laurent; Hervier, Baptiste; Daniel, Laurent; Vuiblet, Vincent; Bardin, Nathalie; Bertin, Daniel; Terrier, Benjamin; Amoura, Zahir; Andrés, Emmanuel; Rondeau, Eric; Hamidou, Mohamed; Pennaforte, Jean-Loup; Halfon, Philippe; Daugas, Eric; Dussol, Bertrand; Puéchal, Xavier; Kaplanski, Gilles; Jourde-Chiche, Noemie

    2016-01-01

    Abstract The aim of the study was to report the clinical, biological, and pathological characteristics of patients with glomerulonephritis (GN) secondary to systemic lupus erythematosus (SLE)/antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) overlap syndrome. A nationwide survey was conducted to identify cases of SLE/AAV overlap syndrome. Data were collected from SLE and AAV French research groups. Inclusion criteria were diagnosis of both SLE and AAV according to international classification criteria and biopsy-proven GN between 1995 and 2014. Additional cases were identified through a systematic literature review. A cohort of consecutive biopsy-proven GN was used to study the prevalence of overlapping antibodies and/or overlap syndrome. The national survey identified 8 cases of SLE/AAV overlap syndrome. All patients were female; median age was 40 years. AAV occurred before SLE (n = 3), after (n = 3), or concomitantly (n = 2). Six patients had rapidly progressive GN and 3/8 had alveolar hemorrhage. All patients had antinuclear antibodies (ANA); 7/8 had p-ANCA antimyeloperoxidase (MPO) antibodies. Renal biopsies showed lupus nephritis (LN) or pauci-immune GN. Remission was obtained in 4/8 patients. A literature review identified 31 additional cases with a similarly severe presentation. In the GN cohort, ANCA positivity was found in 30% of LN, ANA positivity in 52% of pauci-immune GN, with no correlation with pathological findings. The estimated prevalence for SLE/AAV overlap syndrome was 2/101 (2%). In patients with GN, SLE/AAV overlap syndrome may occur but with a low prevalence. Most patients have an aggressive renal presentation, with usually both ANA and anti-MPO antibodies. Further studies are needed to assess shared pathogenesis and therapeutic options. PMID:27258503

  6. Eosinophilic granulomatosis with polyangiitis: an overview

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    Andrea eGioffredi

    2014-11-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA is a multisystemic disorder, belonging to the small vessel ANCA-associated vasculitis, defined as a eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB1*04 and *07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs and silica exposure have been involved.Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells.EGPA is considered a disease with a prevalent activation of the Th2 cellular-mediated inflammatory response but also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g. lungs, heart and gastrointestinal system and the vasculitic phase, characterized by purpura, peripheral neuropathy and constitutional symptoms.ANCA (especially pANCA anti-MPO are present in 40-60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

  7. A study of autoimmune markers in hepatitis C infection.

    Science.gov (United States)

    Agarwal, N; Handa, R; Acharya, S K; Wali, J P; Dinda, A K; Aggarwal, P

    2001-05-01

    Hepatitis C virus (HCV) infection is associated with several autoimmune markers. Despite HCV being common in India, no information on this aspect is available. This study was undertaken to ascertain the frequency and clinical significance of autoimmune markers like rheumatoid factor (RF), antinuclear antibodies (ANA), antibodies to double stranded deoxyribonucleic acid (dsDNA), anti neutrophil cytoplasmic antibody (ANCA), anti smooth muscle antibodies (ASMA), anti liver kidney microsomal 1 antibodies (anti LKM1), anti gastric parietal cell antibodies (anti GPCA), anti mitochondrial antibodies (AMA), anti cardiolipin antibodies (ACL) and cryoglobulins in HCV infection and to determine the effect of treatment on these markers. Twenty five patients with chronic hepatitis C and 25 healthy controls were studied. Cryoglobulins were detected by cryoprecipitation, RF by latex agglutination, anti dsDNA and ACL by ELISA while indirect immunofluorescence was used to detect all other autoantibodies. Eighteen patients (72%) demonstrated autoimmune markers. RF, cryoglobulins and anti LKM1 antibodies were the most frequently detected markers (in 32% patients each). ASMA, perinuclear ANCA (pANCA), ANA and anti GPCA were seen in 24, 20, 12 and 4 per cent patients respectively. None of the patients exhibited ACL, AMA or antibodies to dsDNA. No antibodies were detected in healthy controls. Sixty per cent of the patients had rheumatological symptoms. Of the seven patients followed up after treatment with alpha interferon, only two exhibited persistence of RF, while symptoms and other markers disappeared. Rheumatological symptoms and autoimmune markers are common in HCV infection and are usually overlooked. Patients with unexplained joint pains and/or palpable purpura should be screened for HCV. Further studies are needed to delineate fully the link between infection and autoimmunity.

  8. Study of the diurnal time variability of δ15 N and δ13 C in domestic sewage of Piracicaba city (Sao Paulo State, Brazil)

    International Nuclear Information System (INIS)

    Evangelista, Ricardo A.O.; Bortoletto Junior, Milton J.; Mortatti, Jefferson

    2002-01-01

    Diurnal variability concentrations and δ 13 C of DOC (dissolved organic carbon), DTC (dissolved total carbon) and POC (particulate organic carbon) as well as concentrations and δ 13 N of NH 4 + and PON (particulate organic nitrogen) have been measured using a ANCA-SL mass spectrometry in three points of raw sewage inputs within Piracicaba river. The results shows an important variability in the isotopic signatures and concentrations of the nitrogen and carbon forms during the diurnal period that can be successfully used to characterize and to distinguish three types of investigated effluents. (author)

  9. Mononeuropatia Múltipla como forma de apresentação da Granulomatose Eosinofílica com Poliangeíte

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    Tiago Manuel Fernandes

    2018-03-01

    Full Text Available Resumo: A Granulomatose Eosinofílica com Poliangeíte (EGPA, previamente denominada Síndrome de Churg-Strauss, é uma vasculite associada ao anticorpo anti-citoplasma dos neutrófilos (ANCA que atinge vasos de pequeno calibre. Os autores descrevem o caso de uma mulher com instalação subaguda de parestesias, disestesias, paraparésia crural a impossibilitar a marcha e lesões purpúricas no tornozelo. A electromiografia mostrou mononeuropatia múltipla com envolvimento dos nervos Mediano esquerdo, Cubital e Femoral direitos. Estudo analítico com eosinofilia e ANCA positivo. A biópsia cutânea mostrou vasculite necrotizante e infiltrado eosinofílico. Cumpre a definição de Chapel Hill Conference Consensus e reúne os critérios do American College of Rheumatology pelo que foi assumido o diagnóstico de EGPA. Iniciou corticoterapia e ciclofosfamida, com melhoria clínica e laboratorial. Este caso clínico ilustra a importância do reconhecimento do padrão da neuropatia periférica na apresentação desta entidade rara que pode ser incapacitante se houver atraso no diagnóstico e tratamento. Abstract: Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly known as Churg-Strauss Syndrome, is a anti-neutrophil cytoplasmic antibody (ANCA associated vasculites affecting small vessels. We describe a women with subacute complaints of paresthesias, disesthesias, crural paraparesis with lost of gait and purpuric lesions on her ankle. Electromyogram revealed multiplex mononeuropathy with left median nerve, right ulnar and femoral nerves involvement. Laboratory analysis with eosinophilia and positive ANCA. She fulfilled the Chapel Hill Conference Consensus definition and the criteria of American College of Rheumatology for EGPA. Corticosteroids and cyclophosphamide were begun with good clinical and laboratorial response. This report ilustrates the importance of identifying the neuropatic pattern as initial manifestation of this rare condition

  10. Patogênese e tratamento da glomerulonefrite - uma atualização

    OpenAIRE

    Couser, William G

    2016-01-01

    Resumo A presente revisão traz os conceitos mais atuais acerca dos fatores de risco genéticos, eventos etiológicos, respostas nefritogênicas e tratamento dos principais tipos de glomerulonefrite (GN) imunomediada. Tais patologias incluem GN pós-infecciosa, nefropatia por IgA, doença por anticorpo antimembrana basal glomerular (anti-MBG), vasculite associada a ANCA (VAA) e nefrite lúpica. Apesar da(s) etiologia(s) da maioria dos casos de GN permanecer indefinida, acredita-se que seu início se ...

  11. Proceedings of The 8th Romanian National HIV/AIDS Congress and The 3rd Central European HIV Forum

    OpenAIRE

    Alexiev, Ivailo; Dimitrova, Reneta; Gancheva, Anna; Kostadinova, Asya; Stoycheva, Mariyana; Nikolova, Daniela; Elenkov, Ivaylo; Tili?can, C?t?lin; Predescu, Mioara; P?unescu, Bogdan; Streinu-Cercel, Anca; S?ndulescu, Oana; ?chiopu, Claudiu Mihai; Hristache, M?d?lina; Br?ndu?e, L?cr?mioara Aurelia

    2016-01-01

    O1 HIV-1 diversity in Bulgaria (current molecular epidemiological picture) Ivailo Alexiev, Reneta Dimitrova, Anna Gancheva, Asya Kostadinova, Mariyana Stoycheva, Daniela Nikolova, Ivaylo Elenkov O2 Knowledge, attitudes and practices of the general population on HIV/AIDS, hepatitis B and C in Romania C?t?lin Tili?can, Mioara Predescu, Bogdan P?unescu, Anca Streinu-Cercel, Oana S?ndulescu, Claudiu Mihai ?chiopu, M?d?lina Hristache, L?cr?mioara Aurelia Br?ndu?e, Adrian Streinu-Cercel O3 The prev...

  12. Churg-Strauss syndrome associated with AA amyloidosis: a case report.

    Science.gov (United States)

    Maamar, Mouna; Tazi-Mezalek, Zoubida; Harmouche, Hicham; El Hamany, Zitouna; Adnaoui, Mohammed; Aouni, Mohammed

    2012-01-01

    Churg Strauss syndrome is a rare systemic and pulmonary vasculitis exceptionally associated with AA amyloidosis. We report the case of a 65-year old woman with past medical history of asthma. She developed polyarthralgia, headache and purpura. A laboratory workout found hypereosinophilia (1150/µL), positive p-ANCA, microscopic haematuria and proteinuria at 2g/day. A diagnosis of Churg-Strauss syndrome was established based on five criteria of the American College of Rheumatology (ACR). Renal biopsy showed an important type AA amyloid deposit. The patient was treated with steroids with a good response of the vasculitis and amyloidosis with disappearance of the proteinuria.

  13. Churg-Strauss syndrome: a case with unusual manifestations

    International Nuclear Information System (INIS)

    Restrepo, Mauricio; Gonzalez, Luis Alonso; Vasquez, Gloria

    2008-01-01

    Churg-Strauss syndrome, a necrotizing systemic vasculitis which involves the small and (more rarely) the medium-sized vessels, is a primary vasculitis strongly associated with anti neutrophil cytoplasm antibodies (ANCA). It is characterized by the presence of asthma, eosinophilia and extravascular eosinophilic granulomas. Herein, we report a 36-year-old woman with a history of late onset asthma and allergic rhinitis who developed central nervous system involvement, peripheral neuropathy, leucocytoclastic vasculitis and eosinophilia. Interestingly, unusual clinical manifestations of Churg-Strauss syndrome such as mesenteric micro aneurysms and jaw claudication were present in this particular patient. A brief review of the literature of Churg-Strauss syndrome is presented.

  14. Churg-Strauss syndrome: a case with unusual manifestations; Sindrome de Churg-Strauss: a proposito de un caso con manifestaciones poco usuales

    Energy Technology Data Exchange (ETDEWEB)

    Restrepo, Mauricio; Gonzalez, Luis Alonso; Vasquez, Gloria

    2008-07-01

    Churg-Strauss syndrome, a necrotizing systemic vasculitis which involves the small and (more rarely) the medium-sized vessels, is a primary vasculitis strongly associated with anti neutrophil cytoplasm antibodies (ANCA). It is characterized by the presence of asthma, eosinophilia and extravascular eosinophilic granulomas. Herein, we report a 36-year-old woman with a history of late onset asthma and allergic rhinitis who developed central nervous system involvement, peripheral neuropathy, leucocytoclastic vasculitis and eosinophilia. Interestingly, unusual clinical manifestations of Churg-Strauss syndrome such as mesenteric micro aneurysms and jaw claudication were present in this particular patient. A brief review of the literature of Churg-Strauss syndrome is presented.

  15. A laryngeal presentation of Churg-Strauss syndrome in childhood

    International Nuclear Information System (INIS)

    AlAmmar, Ahmed Y; Yasin, Subhan S; AlMuhsen, Saleh Zaid; AlSaadi Muslim M; AlSohaibanic, Mohammad O

    2009-01-01

    A 10- year-old female, known to have bronchial asthma, presented with an unusual laryngeal lesion, eventually diagnosed as Churg-Strauss syndrome (CSS). She was referred to our hospital with history of recurrent stridor. On endoscopyhe, the larynx showed signs similar to recurrent respiratory papillomatosis (RRP). CSS is a systemic disorder and is now defined as one of the ANCA (antineutrophil cytoplasmic antibodies) - associated vasculitides. CSS is a systemic disease that may involve unusual sites like the laryynx. Such an unusual presenatation of CSS should be kept in mind, especially in patients with history of asthma. (author)

  16. Churg-Strauss Syndrome associated with montelukast: Three Case Reports

    Directory of Open Access Journals (Sweden)

    Fatih Yildiz

    2014-04-01

    Full Text Available Churg-Strauss syndrome (new name Eosinophilic granulomatosis and polyangiitis; asthma, fever, peripheral blood eosinophilia, eosinophilic tissue infiltration, small and medium sized arteries characterized by necrotizing granulomatous inflammation is a multisystemic disorder. Classified in ANCA associated vasculitis. The drugs such as leukotriene receptor antagonists (montelukast, zafirlukast, pranlukast, inhaled glucocorticoids, omalizumab, cocaine and clarithromycin is thought to be associated with Churg-Strauss Syndrome cases have been reported. Herein we presented a rare three CSS cases associated with montelukast. [Cukurova Med J 2014; 39(2.000: 347-352

  17. A laryngeal presentation of Churg-Strauss syndrome in childhood

    Energy Technology Data Exchange (ETDEWEB)

    AlAmmar, Ahmed Y; Yasin, Subhan S; AlMuhsen, Saleh Zaid [Dept. of Otolaryngology, Head and Neck Surgery, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia); M, AlSaadi Muslim [Dept. of Pediatrics, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia); AlSohaibanic, Mohammad O [Dept. of Pathology, King Abdulaziz Univ. Hospital, Riyadh (Saudi Arabia)

    2009-07-01

    A 10- year-old female, known to have bronchial asthma, presented with an unusual laryngeal lesion, eventually diagnosed as Churg-Strauss syndrome (CSS). She was referred to our hospital with history of recurrent stridor. On endoscopyhe, the larynx showed signs similar to recurrent respiratory papillomatosis (RRP). CSS is a systemic disorder and is now defined as one of the ANCA (antineutrophil cytoplasmic antibodies) - associated vasculitides. CSS is a systemic disease that may involve unusual sites like the laryynx. Such an unusual presenatation of CSS should be kept in mind, especially in patients with history of asthma. (author)

  18. Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome as a differential diagnosis of hypereosinophilic syndromes

    Directory of Open Access Journals (Sweden)

    Yuri Albuquerque Pessoa Santos

    2017-01-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs and hypereosinophilic syndromes (HES. Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes.

  19. Membranous nephropathy associated with familial chronic ulcerative colitis in a 12-year-old girl.

    Science.gov (United States)

    Ridder, Regina M; Kreth, Hans W; Kiss, Eva; Gröne, Hermann J; Gordjani, Nader

    2005-09-01

    Glomerulonephritis is a rare complication in patients with inflammatory bowel disease. We report a case of membranous nephropathy (MN) in a 12.6-year-old girl with chronic ulcerative colitis. The girl was referred to the hospital with bloody diarrhea and arthralgia. Routine urinalysis showed 1 g/m(2) protein excretion in 24 h. Serum ANCA titers were positive. The diagnoses were confirmed by coloscopy and kidney biopsy. The patient's mother had also suffered from ulcerative colitis in adolescence. Proteinuria normalized under treatment with prednisone (60 mg/m(2)/day) and azathioprine, which was initiated to treat the colitis. Chronic ulcerative colitis can be associated with glomerulonephritis.

  20. Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos

    OpenAIRE

    Marcelo Fernández Casares; Alejandra González; Flavia Caputo; Yanina Bottinelli; Patricia Nastavi; Marcelo Zamboni

    2012-01-01

    Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA) positivos (VAA), son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP), sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográfi...

  1. Microscopic polyangiitis associated with primary biliary cirrhosis, Sjogren′s syndrome and Hashimoto′s thyroiditis

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    I Ben Ghorbel

    2015-01-01

    Full Text Available The association between microscopic polyangiitis (MPA and primary biliary cirrhosis (PBC has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren′s syndrome and Hashimoto′s thyroiditis.

  2. Exploring the novel heterocyclic derivatives as lead molecules for design and development of potent anticancer agents.

    Science.gov (United States)

    Azad, Iqbal; Nasibullah, Malik; Khan, Tahmeena; Hassan, Firoj; Akhter, Yusuf

    2018-05-01

    This paper deals with in silico evaluation of newly proposed heterocyclic derivatives in search of potential anticancer activity. Best possible drug candidates have been proposed using a rational approach employing a pipeline of computational techniques namely MetaPrint2D prediction, molinspiration, cheminformatics, Osiris Data warrior, AutoDock and iGEMDOCK. Lazar toxicity prediction, AdmetSAR predictions, and targeted docking studies were also performed. 27 heterocyclic derivatives were selected for bioactivity prediction and drug likeness score on the basis of Lipinski's rule, Viber rule, Ghose filter, leadlikeness and Pan Assay Interference Compounds (PAINS) rule. Bufuralol, Sunitinib, and Doxorubicin were selected as reference standard drug for the comparison of molecular descriptors and docking. Bufuralol is a known non-selective adreno-receptor blocking agent. Studies showed that beta blockers are also used against different types of cancers. Sunitinib is well known Food and Drug administration (FDA) approved pyrrole containing tyrosine kinase inhibitor and our proposed molecules possess similarities with both drug and doxorubicin is another moiety having anticancer activity. All heterocyclic derivatives were found to obey the drug filters except standard drug Doxorubicin. Bioactivity score of the compounds was predicted for drug targets including enzymes, nuclear receptors, kinase inhibitors, G protein-coupled receptor (GPCR) ligands and ion channel modulators. Absorption, distribution, metabolism and toxicity (ADMET) prediction of all proposed compound showed good Blood-brain barrier (BBB) penetration, Human intestinal absorption (HIA), Caco-2 cell permeability except compound-11 and was found to have no AdmetSAR toxicity as well as carcinogenic effect. Compounds 1-9 were slightly mutagenic while compound 2, 11, 20 and 21 showed carcinogenic effect according to Lazar toxicity prediction. Rests of the compounds were predicted to have no side effect

  3. Systemic Vasculitis During the Course of Systemic Sclerosis

    Science.gov (United States)

    Quéméneur, Thomas; Mouthon, Luc; Cacoub, Patrice; Meyer, Olivier; Michon-Pasturel, Ulrique; Vanhille, Philippe; Hatron, Pierre-Yves; Guillevin, Loïc; Hachulla, Eric

    2013-01-01

    Abstract Although the presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients with systemic sclerosis (SSc), the association of SSc and systemic vasculitis has rarely been described. We obtained information on cases of systemic vasculitis associated with SSc in France from the French Vasculitis Study Group and all members of the French Research Group on Systemic Sclerosis. We identified 12 patients with systemic vasculitis associated with SSc: 9 with ANCA-associated systemic vasculitis (AASV) and 3 with mixed cryoglobulinemia vasculitis (MCV). In all AASV patients, SSc was of the limited type. The main complication of SSc was pulmonary fibrosis. Only 2 patients underwent a D-penicillamine regimen before the occurrence of AASV. The characteristics of AASV were microscopic polyangiitis (n = 7) and renal limited vasculitis (n = 2). Anti-myeloperoxidase antibodies were found in 8 of the 9 patients. The Five Factor Score was above 1 in 3 of the 9 patients. Of the 3 patients with MCV, Sjögren syndrome was confirmed in 2. We compared our findings with the results of a literature review (42 previously reported cases of AASV with SSc). Although rare, vasculitis is a complication of SSc. AASV is the most frequent type, and its diagnosis can be challenging when the kidney is injured. Better awareness of this rare association could facilitate earlier diagnosis and appropriate management to reduce damage. PMID:23263715

  4. Biomarkers in Vasculitis

    Science.gov (United States)

    Monach, Paul A.

    2014-01-01

    Purpose of review Better biomarkers are needed for guiding management of patients with vasculitis. Large cohorts and technological advances had led to an increase in pre-clinical studies of potential biomarkers. Recent findings The most interesting markers described recently include a gene expression signature in CD8+ T cells that predicts tendency to relapse or remain relapse-free in ANCA-associated vasculitis, and a pair of urinary proteins that are elevated in Kawasaki disease but not other febrile illnesses. Both of these studies used “omics” technologies to generate and then test hypotheses. More conventional hypothesis-based studies have indicated that the following circulating proteins have potential to improve upon clinically available tests: pentraxin-3 in giant cell arteritis and Takayasu’s arteritis; von Willebrand factor antigen in childhood central nervous system vasculitis; eotaxin-3 and other markers related to eosinophils or Th2 immune responses in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome); and MMP-3, TIMP-1, and CXCL13 in ANCA-associated vasculitis. Summary New markers testable in blood and urine have the potential to assist with diagnosis, staging, assessment of current disease activity, and prognosis. However, the standards for clinical usefulness, in particular the demonstration of either very high sensitivity or very high specificity, have yet to be met for clinically relevant outcomes. PMID:24257367

  5. Infliximab en pacientes con enfermedad ocular inflamatoria, refractarios a DARMES

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    Elmer R. García-Salazar

    2013-07-01

    Full Text Available Se describe la experiencia con infliximab (anticuerpo monoclonal con una potente acción antiinflamatoria en el tratamiento de enfermedades oculares inflamatorias secundarias a patologías reumáticas y refractarias a drogas antirreumáticas modificadoras de la enfermedad (DARMES. Se evaluó el caso de una paciente de 50 años con artritis reumatoide (AR de fondo activo y una paciente de 37 años con vasculitis anticuerpos anticitoplasma de neutrófilos especifico para mieloperoxidasa (ANCA MPO sin compromiso de órgano noble, ambas con escleritis bilateral y perforación con prolapso de iris del ojo izquierdo. Ellas recibieron infliximab EV en dosis de 3 a 5 mg/kg/dosis, según el esquema, a las 0, 2, 6 y 8 semanas. Infliximab resultó eficaz y seguro para el tratamiento de escleritis asociada a AR y vasculitis ANCA MPO positivo, refractaria a tratamiento con DARMES y corticoides en dosis altas. Los injertos de tejido esclerocorneal evolucionaron favorablemente con infliximab.

  6. Pauci-immune crescentic glomerulonephritis in the Down′s syndrome

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    Mejda Cherif

    2013-01-01

    Full Text Available Kidney disease is a rare complication in patients with the Down′s syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephritis with hypo-complementemia, crescentic glomerulonephritis with anti-neutrophil cytoplasmic antibodies (ANCA, amyloidosis and immunotactoid glomerulopathy. We report the observation of a 38-year-old man with the Down′s syndrome who presented with severe renal failure, proteinuria and microscopic hematuria evolving over two months. There was no history of congenital heart disease or urinary symptoms. Percutaneous renal biopsy revealed fibrous crescents, rupture of Bowman′s capsule and peri-glomerular granuloma; there were no deposits on immunofluorescence study. Thoracic computerized tomography scan showed alveolar congestion. The patient tested negative for ANCA. At the time of reporting, the patient is on regular chronic hemodialysis. Our case illustrates a distinct entity that further expands the spectrum of renal disease known to occur in the Down′s syndrome. Early detection of the renal disorders may prevent or slow down the progression.

  7. Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis.

    Science.gov (United States)

    Pajor, Anna Maria; Kwiatkowska, Sylwia; Kroczyńska-Bednarek, Jadwiga; Piotrowski, Wojciech J

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

  8. Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

    Science.gov (United States)

    Groh, Matthieu; Masciocco, Gabriella; Kirchner, Elizabeth; Kristen, Arnt; Pellegrini, Carlo; Varnous, Shaïda; Bortman, Guillermo; Rosenberg, Mark; Brucato, Antonio; Waterworth, Paul; Bonacina, Edgardo; Facchetti, Fabio; Calabrese, Leonard; Gregorini, Gina; Scali, Juan Jose; Starling, Randall; Frigerio, Maria; D'Armini, Andrea Maria; Guillevin, Loïc

    2014-08-01

    Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once. We conducted a retrospective international multicenter study. Patients satisfying the criteria of the American College of Rheumatology and/or revised Chapel Hill Consensus Conference Nomenclature were identified by collaborating vasculitis and transplant specialists, and the help of the Churg-Strauss Syndrome Association. Nine ANCA(-) patients who received transplants between October 1987 and December 2009 were identified. The vasculitis and cardiomyopathy diagnoses were concomitant for 5 patients and separated by 12 to 288 months for the remaining 4 patients. Despite ongoing immunosuppression, histologic examination of 7 (78%) patients' explanted hearts showed histologic patterns suggestive of active vasculitis. The overall 5-year survival rate was low (57%), but rose to 80% when considering only the 6 patients transplanted during the last decade. After survival lasting 3 to 60 months, 4 (44%) patients died sudden deaths. The search for EGPA-related cardiomyopathy is mandatory early in the course of this type of vasculitis. Indeed, prompt treatment with corticosteroids and cyclophosphamide may achieve restore cardiac function. Most patients in this series were undertreated. For patients with refractory EGPA, heart transplantation should be performed, which carries a fair prognosis. No optimal immunosuppressive strategy has yet been identified. Copyright © 2014 International Society for Heart and Lung Transplantation. All rights reserved.

  9. Successful management of Churg-Strauss syndrome using omalizumab as adjuvant immunomodulatory therapy: first documented pediatric case.

    Science.gov (United States)

    Iglesias, E; Camacho Lovillo, M; Delgado Pecellín, I; Lirola Cruz, M J; Falcón Neyra, M D; Salazar Quero, J C; Bernabeu-Wittel, J; González Valencia, J P; Neth, O

    2014-03-01

    Churg-Strauss syndrome (CSS) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis; it is extremely rare in childhood and defined according to the Chapel-Hill Consensus as an eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. Children commonly have a history of asthma and sinusitis whilst clinical presentation typically involves pulmonary tract and less frequently skin, heart, gastrointestinal tract, and peripheral nerves. Cardiopulmonary disease is higher in children and prognosis is worse. It is associated with significant eosinophilia and raised serum IgE-levels. ANCA are only found in 25% of childhood cases. Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis. She was treated with corticosteroids, cyclophosphamide, intravenous immunoglobulin, mycophenolate mofetil (MMF), and rituximab. However, remission was only achieved after initiation of omalizumab therapy, a recombinant humanized anti-IgE antibody. To the best of our knowledge this is the first pediatric patient suffering from CSS successfully managed with adjuvant anti-IgE therapy resulting in the control of respiratory as well as gastrointestinal symptoms. © 2013 Wiley Periodicals, Inc.

  10. Síndroma de Churg-Strauss – a propósito de um caso clínico

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    Susana Oliveira

    2005-01-01

    Full Text Available Resumo: A síndroma de Churg-Strauss (SCS é uma vasculite sistémica pouco comum, que atinge preferencialmente vasos de pequeno calibre, envolve múltiplos órgãos e surge quase sempre associada a asma e eosinofilia.Os autores fazem uma revisão sobre esta doença e discutem o seu diagnóstico diferencial a propósito do caso clínico de uma mulher com diagnóstico de asma brônquica que aos 65 anos inicia quadro compatível com SCS.Rev Port Pneumol 2005; XI (1: 73-83 Abstract: Churg-Strauss syndrome (CSS is a rare systemic vasculitis, that essentially affects medium-sized vessels, involves multiple organs and in the majority of cases is related to asthma and eosinophilia.The authors make a review of this disease and discuss its differential diagnosis presenting the case of a woman with asthma that at the age of 65 is diagnosed with CSS.Rev Port Pneumol 2005; XI (1: 73-83 Palavras-Chave: Churg-Strauss, asma, vasculite, ANCA, Key words: Churg-Strauss, asthma, vasculitis, ANCA

  11. Efficiency of using rituximab in a patient with generalized granulomatosis with polyangiitis: A case report

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    Gulazyk Malikovna Koilubaeva

    2014-01-01

    Full Text Available Systemic vasculitides (SVs are characterized by inflammation of the blood vessels wall; the spectrum of their clinical manifestations depends on the type, extent, and location of affected vessels and the activity of systemic inflammation. The etiology of most primary SVs is unknown. Antineutrophil cytoplasmic antibodies (ANCAs are implicated in its pathogenesis. The presence of ANCAa in patients' serum and the correlation of their level with the severity of clinical manifestations served as a basis for identifying a subgroup of systemic necrotizing vasculitides associated with ANCA synthesis: granulomatosis with polyangiitis (GPA, microscopic polyangiitis, and Churg – Strauss syndrome. GPA is characterized by systemic granulomatous necrotizing vasculitis involving the small vessels of the upper respiratory tract, lung, and kidney.The paper describes a case of difficult diagnosis and successful rituximab (RTM treatment of generalized GPA in a 45-year-old female patients. The disease occurred with local damage to the upper respiratory tract, granulomatous inflammation of the pulmonary vessels to form multiple infiltrates with lung tissue destruction elements and necrotizing glomerulonephritis. Despite intensive immunosuppressive treatment, there was a rapid GPA progression with the further development of respiratory failure, which had been induced by stenotic laryngitis subglottica leading to tracheostoma. Damage to the organ of vision could lead to severe complications, including amaurosis. RMT was shown to be effective in treating generalized GPA with a poor prognosis.

  12. Atualização do tratamento das vasculites associadas a anticorpo anticitoplasma de neutrófilos Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: update

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    Alfredo Nicodemos Cruz Santana

    2011-12-01

    Full Text Available As vasculites antineutrophil cytoplasmic antibody (ANCA, anticorpo anticitoplasma de neutrófilos associadas (VAAs são caracterizadas por uma inflamação sistêmica das artérias de pequeno e médio calibre (especialmente no trato respiratório superior e inferior, e nos rins. As VAAs compreendem a granulomatose de Wegener (agora chamada de granulomatose com poliangeíte, poliangeíte microscópica, VAA limitada ao rim e a síndrome de Churg-Strauss. Neste artigo, discutiremos as fases de tratamento dessas vasculites, como fase de indução (com ciclofosfamida ou rituximab e fase de manutenção (com azatioprina, metotrexato ou rituximab. Além disso, discutiremos como manusear os casos refratários à ciclofosfamida.In its various forms, antineutrophil cytoplasmic antibody (ANCA-associated vasculitis (AAV is characterized by a systemic inflammation of the small and medium-sized arteries (especially in the upper and lower respiratory tracts, as well as in the kidneys. The forms of AAV comprise Wegener's granulomatosis (now called granulomatosis with polyangiitis, microscopic polyangiitis, renal AAV, and Churg-Strauss syndrome. In this paper, we discuss the phases of AAV treatment, including the induction phase (with cyclophosphamide or rituximab and the maintenance phase (with azathioprine, methotrexate, or rituximab. We also discuss how to handle patients who are refractory to cyclophosphamide.

  13. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome and pulmonary thromboembolism: an overlooked concomitance

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    Vilma Takayasu

    2013-06-01

    Full Text Available The Eosinophilic Granulomatosis with Polyangiitis (formerly Churg- Strauss Syndrome (EGPA is a systemic inflammatory disease characterized by the presence of rhinitis, asthma, peripheral eosinophilia, and vasculitis—the latter being characteristic of the late stage of the disease. After several years from the onset of the disease, small- and medium-sized vessel vasculitis ensues, undertaking various organs and systems. Upper and lower airways, skin, nervous system, gastrointestinal tract, heart, and kidneys are the most commonly involved organs. It is believed that tissue injury is the result of processes mediated by antineutrophil cytoplasmic antibody (ANCA, or toxic mediators released by eosinophils. Although it is classified as ANCA-associated vasculitis, these autoantibodies are present in only 40% of cases. The authors report the case of a patient with EGPA, who had a history of asthma, peripheral and central neuropathy, palpable purpura, gastrointestinal micro perforation, peripheral eosinophilia, and the presence of myeloperoxidase-antineutrophil cytoplasmic antibody. Inflammatory parameters improved after the initiation of treatment, but 1 month after hospital discharge the patient developed symptoms compatible with pulmonary embolism and died. Thrombophilia that occurs in EGPA is due to the interaction between the inflammatory response and eosinophilia with the clotting system resulting in a pro-thrombotic state. Although not yet well-determined, the authors call attention to the possibility of the impact of thromboembolic events on the prognosis of patients with EGPA. In addition to the adequate immunosuppressive treatment, prophylaxis and treatment for thrombosis should never be overlooked.

  14. Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos

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    Marcelo Fernández Casares

    2012-08-01

    Full Text Available Las complicaciones pulmonares más conocidas de las vasculitis con anticuerpos anticitoplasmáticos de los neutrófilos (ANCA positivos (VAA, son la hemorragia alveolar, los granulomas y la estenosis de la vía aérea. En los últimos años han aparecido algunos informes aislados que muestran la asociación con fibrosis pulmonar (FP, sugiriendo que ésta sería otra complicación de las VAA. En este trabajo informamos dos casos con dicha asociación describiendo sus características clínicas, tomográficas e inmunológicas. Dado que en la asociación de FP y VAA notificada en los últimos años, la FP puede ser su primera manifestación, podría ser necesaria la búsqueda de ANCA en pacientes con FP, como causa de la misma y por el posible desarrollo posterior de vasculitis.

  15. Breaking down the complement system: a review and update on novel therapies.

    Science.gov (United States)

    Reddy, Yuvaram N V; Siedlecki, Andrew M; Francis, Jean M

    2017-03-01

    The complement system represents one of the more primitive forms of innate immunity. It has increasingly been found to contribute to pathologies in the native and transplanted kidney. We provide a concise review of the physiology of the complement cascade, and discuss current and upcoming complement-based therapies. Current agents in clinical use either bind to complement components directly or prevent complement from binding to antibodies affixed to the endothelial surface. These include C1 esterase inhibitors, anti-C5 mAbs, anti-CD20 mAbs, and proteasome inhibitors. Treatment continues to show efficacy in the atypical hemolytic uremic syndrome and antibody-mediated rejection. Promising agents not currently available include CCX168, TP10, AMY-101, factor D inhibitors, coversin, and compstatin. Several new trials are targeting complement inhibition to treat antineutrophilic cystoplasmic antibody (ANCA)-associated vasculitis, C3 glomerulopathy, thrombotic microangiopathy, and IgA nephropathy. New agents for the treatment of the atypical hemolytic uremic syndrome are also in development. Complement-based therapies are being considered for targeted therapy in the atypical hemolytic uremic syndrome and antibody-mediated rejection, C3 glomerulopathy, and ANCA-associated vasculitis. A few agents are currently in use as orphan drugs. A number of other drugs are in clinical trials and, overall, are showing promising preliminary results.

  16. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy

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    Ryan Kunjal

    2016-01-01

    Full Text Available Antineutrophil cytoplasmic antibody (ANCA associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN. The diagnosis was consistent with granulomatosis with polyangiitis (GPA. Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

  17. Granulomatosis with Polyangiitis Presenting as Pauci-Immune Crescentic Glomerulonephritis in Pregnancy.

    Science.gov (United States)

    Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management.

  18. [Surgical treatment with dacryocystitis and retinal detachment in a patient with Wegener granulomatosis].

    Science.gov (United States)

    Metoki, Tomomi; Kubo, Masabumi; Takano, Yoshiko; Nakamura, Hideo; Nakazawa, Mitsuru

    2003-06-01

    We report surgical treatment of a patient with dacryocystitis and retinal detachment (RD), which are rare ophthalmic involvements of Wegener granulomatosis (WG). The patient was a 26-year-old man with WG. He was diagnosed as having WG 4 years ago and he has been treated by maintenance doses of predonisolone and cyclophosphamide. Rheumatoid factor and serum antinuclear antibody were negative. Cytoplasmic pattern-antineutrophil cytoplasmic antibody (C-ANCA) and renal function were normal. He was found to have nasolacrimal duct obstruction and lattice degeneration bilaterally, retinal tear with RD in the left eye and tear without RD in the right eye. No sign of vasculitis was found in fluorescein angiography. Bilateral dacryocystorhinostomy was performed without any sign of postoperative necrosis of the wound. After the surgery, epiphora and eye discharge disappeared and lacrimal passage has been maintained without obstruction. The pathological findings of his nasal mucosa and lacrimal sac showed chronic inflammation and no typical changes of WG. There was no abnormal change in the conjunctiva and sclera after an uncomplicated scleral buckling surgery. We conclude that operations such as dacryocystorhinostomy and scleral buckling surgery may be performed successfully when WG is controlled within the normal limits of C-ANCA.

  19. [Diverse histological lesions in a patient with antiphospholipid syndrome (APS)].

    Science.gov (United States)

    Salvatore, Ermanno; Luciani, Remo; Di Palma, Annamaria; Aversano, Arturo; Stellato, Davide; Liuzzi, Marco; Iele, Emilio; Martignetti, Vinicio; Spagnuolo, Enrico; Morrone, Luigi

    2011-01-01

    Antiphospholipid syndrome (APS) is a rare autoimmune disorder. It can be secondary to systemic lupus erythematosus (SLE) or occur in the absence of autoimmune disease. The hallmark of this so-called primary APS is the presence of circulating antiphospholipid antibodies. Renal involvement in primary APS is caused by thrombosis within the renal vasculature. Recently, nonthrombotic glomerulonephritic renal lesions have been described in primary APS as a new histological entity. We here report a patient with primary APS in whom both lesion types were present. A 58-year-old Caucasian man with no significant past medical history presented to our nephrology unit with diffuse edema. Urinalysis showed proteinuria exceeding 400 mg/dL. The autoantibody panel (p-ANCA, c- ANCA, anti-nucleus, anti-DS-DNA) was negative except for anticardiolipin antibodies, which tested positive in two different samples. The diagnostic workup included a kidney biopsy that revealed thrombotic lesions compatible with primary APS and a typical pattern of focal segmental glomerulosclerosis. The kidney is a major target in APS but the exact mechanism underlying the pathogenesis of APS nephropathy has been poorly recognized. The use of kidney biopsy is a fundamental diagnostic tool in this setting, with possible implications also from a prognostic and therapeutic viewpoint.

  20. Autoimmune Thyroiditis and Glomerulopathies

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    Domenico Santoro

    2017-06-01

    Full Text Available Autoimmune thyroiditis (AIT is generally associated with hypothyroidism. It affects ~2% of the female population and 0.2% of the male population. The evidence of thyroid function- and thyroid autoantibody-unrelated microproteinuria in almost half of patients with AIT and sometimes heavy proteinuria as in the nephrotic syndrome point to a link of AIT with renal disease. The most common renal diseases observed in AIT are membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic autoantibody (ANCA vasculitis, and amyloidosis. Different hypotheses have been put forward regarding the relationship between AIT and glomerulopathies, and several potential mechanisms for this association have been considered. Glomerular deposition of immunocomplexes of thyroglobulin and autoantibodies as well as the impaired immune tolerance for megalin (a thyrotropin-regulated glycoprotein expressed on thyroid cells are the most probable mechanisms. Cross-reactivity between antigens in the setting of genetic predisposition has been considered as a potential mechanism that links the described association between ANCA vasculitis and AIT.

  1. Detection of anti-lactoferrin antibodies and anti-myeloperoxidase antibodies in autoimmune hepatitis: a retrospective study.

    Science.gov (United States)

    Tan, Liming; Zhang, Yuhong; Peng, Weihua; Chen, Juanjuan; Li, Hua; Ming, Feng

    2014-01-01

    Anti-lactoferrin antibodies (ALA) and anti-myeloperoxidase antibodies (AMPA) are specific serological markers for autoimmune hepatitis (AIH). The project aimed to detect ALA and AMPA and explore their clinical significances in AIH patients. 59 AIH patients, 217 non AIH patients, and 50 healthy controls were enrolled in this study. ALA and AMPA were detected by ELISA. Antineutropil cytoplasmic antibodies (ANCA) and anti-smooth muscle antibodies (ASMA) were examined by indirect immunofluorescence. Antimitochondrial antibody M2 subtype (AMA-M2), anti-liver kidney microsomal antibody Type 1 (LKM1), anti-liver cytosol antibody Type 1 (LC1), and anti-soluble liver antigen/liver-pancreas antibodies (SLA/LP) were tested by immunoblot. The positivity for ALA was 18.6% in AIH group, only one patient in non-AIH group was positive for ALA; the positivity for AMPA was 59.3% in AIH group, with significant differences (P < 0.01) compared with other groups. The specificities for ALA and AMPA were 99.63% and 97.75%; the sensitivities were 18.64% and 59.32%; and the accuracy rates were 84.97% and 90.80%, respectively. A certain correlation was observed between ALA and SLA/LP, AMPA and ANCA, ASMA in AIH group. ALA and AMPA were associated with AIH, and had high clinical diagnostic value. Co-detection with other relative autoantibodies could play an important role in differential diagnosis of AIH.

  2. New serological markers in pediatric patients with inflammatory bowel disease

    Science.gov (United States)

    Kovács, Márta; Müller, Katalin Eszter; Papp, Mária; Lakatos, Péter László; Csöndes, Mihály; Veres, Gábor

    2014-01-01

    The spectrum of serological markers associated with inflammatory bowel disease (IBD) is rapidly growing. Due to frequently delayed or missed diagnoses, the application of non-invasive diagnostic tests for IBD, as well as differentiation between ulcerative colitis (UC) and Crohn’s disease (CD), would be useful in the pediatric population. In addition, the combination of pancreatic autoantibodies and antibodies against Saccharomyces cerevisiae antibodies/perinuclear cytoplasmic antibody (pANCA) improved the sensitivity of serological markers in pediatric patients with CD and UC. Some studies suggested that age-associated differences in the patterns of antibodies may be present, particularly in the youngest children. In CD, most patients develop stricturing or perforating complications, and a significant number of patients undergo surgery during the disease course. Based on recent knowledge, serum antibodies are qualitatively and quantitatively associated with complicated CD behavior and CD-related surgery. Pediatric UC is characterized by extensive colitis and a high rate of colectomy. In patients with UC, high levels of anti-CBir1 and pANCA are associated with the development of pouchitis after ileal pouch-anal anastomosis. Thus, serologic markers for IBD can be applied to stratify IBD patients into more homogeneous subgroups with respect to disease progression. In conclusion, identification of patients at an increased risk of rapid disease progression is of great interest, as the application of early and more aggressive pharmaceutical intervention could have the potential to alter the natural history of IBD, and reduce complications and hospitalizations. PMID:24803798

  3. Including spatial data in nutrient balance modelling on dairy farms

    Science.gov (United States)

    van Leeuwen, Maricke; van Middelaar, Corina; Stoof, Cathelijne; Oenema, Jouke; Stoorvogel, Jetse; de Boer, Imke

    2017-04-01

    The Annual Nutrient Cycle Assessment (ANCA) calculates the nitrogen (N) and phosphorus (P) balance at a dairy farm, while taking into account the subsequent nutrient cycles of the herd, manure, soil and crop components. Since January 2016, Dutch dairy farmers are required to use ANCA in order to increase understanding of nutrient flows and to minimize nutrient losses to the environment. A nutrient balance calculates the difference between nutrient inputs and outputs. Nutrients enter the farm via purchased feed, fertilizers, deposition and fixation by legumes (nitrogen), and leave the farm via milk, livestock, manure, and roughages. A positive balance indicates to which extent N and/or P are lost to the environment via gaseous emissions (N), leaching, run-off and accumulation in soil. A negative balance indicates that N and/or P are depleted from soil. ANCA was designed to calculate average nutrient flows on farm level (for the herd, manure, soil and crop components). ANCA was not designed to perform calculations of nutrient flows at the field level, as it uses averaged nutrient inputs and outputs across all fields, and it does not include field specific soil characteristics. Land management decisions, however, such as the level of N and P application, are typically taken at the field level given the specific crop and soil characteristics. Therefore the information that ANCA provides is likely not sufficient to support farmers' decisions on land management to minimize nutrient losses to the environment. This is particularly a problem when land management and soils vary between fields. For an accurate estimate of nutrient flows in a given farming system that can be used to optimize land management, the spatial scale of nutrient inputs and outputs (and thus the effect of land management and soil variation) could be essential. Our aim was to determine the effect of the spatial scale of nutrient inputs and outputs on modelled nutrient flows and nutrient use efficiencies

  4. A history of the work concept from physics to economics

    CERN Document Server

    Oliveira, Agamenon R E

    2014-01-01

    This  book traces the history of the concept of work from its earliest stages and shows that its further formalization leads to equilibrium principle and to the principle of virtual works, and so pointing the way ahead for future research and applications. The idea that something remains constant in a machine operation is very old and has been expressed by many mathematicians and philosophers such as, for instance, Aristotle. Thus,  a concept of energy developed. Another important  idea in machine operation is Archimedes' lever principle. In modern times the concept of work is analyzed in the context of applied mechanics mainly in Lazare Carnot mechanics and the mechanics of the new generation of polytechnical engineers like Navier, Coriolis and Poncelet. In this context the word "work" is finally adopted. These engineers are also responsible for the incorporation of the concept of work into the discipline of economics when they endeavoured to combine the study  of the work of machines and men together.

  5. Europe’s Southeastern Gateway: Romania’s Public Policy Respose to Changing Patterns of World Shipping

    Directory of Open Access Journals (Sweden)

    Roger HAMLIN

    2015-06-01

    Full Text Available This article is a follow-up to one published in December of 2012 (Hamlin and Lazar, 2012. Global trade and related transportation are changing dramatically. Trends are diff cult to fol-low, but important in the effect on cities, coun-tries and continents. Over the past two decades, rising energy prices, rising wages, environmen-tal concerns and other factors have produced a shift back to ocean shipping as an important transportation mode. While slower than other modes, ocean freighter transport can be lower in cost and create lower carbon emissions. These advantages continue to improve as container freighters are becoming larger. The purpose of the article is to look more closely at the public and private sector response in the key Romania port of Constanţa. The f rst part will lay out the current situation. The second will update and evaluate the Eastern European responses to the current situation, looking closely at the Port of Constanţa.

  6. Process of long-term tunnel instability by temperature and humidity variation in sedimentary rock

    International Nuclear Information System (INIS)

    Sawada, Masataka; Okada, Tetsuji; Nakata, Eiji

    2009-01-01

    It is concerned that tunnels in the sedimentary rock are seriously damaged during the long operation after excavation, while there are various plans to construct significant underground facilities such as a high-level radioactive waste disposal facility. A case history study on tunnel instability is important in order to assess and evaluate tunnel instability behavior. In this respect, an accelerated tunnel deformation test by removing tunnel supports was conducted. Instability of tunnel wall was observed before and after this test in the summer, when it is warm and humid in the test tunnel. Fiber optic sensing detected the instability. Scale of collapsed rock was evaluated from the variation of shape of tunnel cross-section measured by a 3-D lazar measurement tool. The maximum size of collapsed rock block is 1m in diameter. Surrounding sandstone has such a characteristic that crack growth is much faster and its strength decreases gradually in the condition of high relative humidity. Numerical simulation considering this decrease of rock strength reproduced the instable zone around the test tunnel. (author)

  7. The 1 Repetition Maximum Mechanics of a High-Handle Hexagonal Bar Deadlift Compared With a Conventional Deadlift as Measured by a Linear Position Transducer.

    Science.gov (United States)

    Lockie, Robert G; Moreno, Matthew R; Lazar, Adrina; Risso, Fabrice G; Liu, Tricia M; Stage, Alyssa A; Birmingham-Babauta, Samantha A; Torne, Ibett A; Stokes, John J; Giuliano, Dominic V; Davis, DeShaun L; Orjalo, Ashley J; Callaghan, Samuel J

    2018-01-01

    Lockie, RG, Moreno, MR, Lazar, A, Risso, FG, Liu, TM, Stage, AA, Birmingham-Babauta, SA, Torne, IA, Stokes, JJ, Giuliano, DV, Davis, DL, Orjalo, AJ, and Callaghan, SJ. The 1 repetition maximum mechanics of a high-handle hexagonal bar deadlift compared with a conventional deadlift as measured by a linear position transducer. J Strength Cond Res 32(1): 150-161, 2018-The high-handle hexagonal bar deadlift (HHBD), a variation of the conventional deadlift (CD), is said to reduce the lift range of motion, which may change the mechanics of the lift. However, no research has investigated this. This study compared the mechanics between a 1 repetition maximum (1RM) CD and HHBD. Thirty-one strength-trained subjects (21 men, 10 women) completed a 1RM CD and HHBD. A linear position transducer measured lift distance, duration, and work; and peak and mean power, velocity, and force. The presence of a sticking region (SR) was determined for each lift. A repeated-measures analysis of variance (ANOVA) calculated differences between 1RM CD and HHBD mechanics. A one-way ANOVA compared the mechanics of each lift between subjects who exhibited an SR or not, and the SR between the CD and HHBD. Significance was set at p mechanics between subjects with or without an SR, and no differences in SR region distance or duration between the CD and HHBD. Greater force can be generated in the HHBD, which could have implications for strength-training adaptations over time.

  8. ALEXA VISARION AND THE CARAGIALIAN TEXTS - ESSENCE AND EXPRESSIVENESS

    Directory of Open Access Journals (Sweden)

    BĂLĂIŢĂ VASILICA

    2015-09-01

    Full Text Available Ion Luca Caragiale is a national playwright, and is considered a symbol of Romanian society, and more than 100 years have passed since apparently we have not separated psychologically and behaviourally from the template (pattern in which loan Luca Caragiale had set the Romanian society. We believe it’s even worse that students at the Faculty of Theater, in the ab­sence of anthropological study, play dramatic texts literally, which increases the risk to stereotype Caragiale’s opera in school. In this article we intend to present Alexa Visarion’s[1] films, based on Ion Luca Caragiale’s short stories, as well as the play Dale Carnavalului (Of the Carnival directed by him, as a guest professor, at the National Theatre Art Institute from Reykja-vic, Island (1989. The movies we refer to were the result of some performances played almost with the same distribution for a decade: Năpasta (The Scourage, after the short story with the same name, a movie directed in 1985, starring Dorina Lazar, Florin Zamfirescu and Dorel Visan; Inainte de tacere (Before silence, directed in 1978, after the story In vreme de razboi (in time of war novel starring Valeria Seciu and Liviu Rozorea. Our demarche aims at highlighting the universality of Ioan Luca Caragiale by essentialization and expressivity.

  9. Cómo hacer necropolíticas en casa: Ideología de género y acuerdos de paz en Colombia

    Directory of Open Access Journals (Sweden)

    Camila Esguerra Muelle

    Full Text Available Resumen Este artículo propone el análisis de un acervo de discursos audiovisuales y escritos difundidos por sectores que fomentaron la votación por el “no” en el plebiscito por la paz, realizado el 2 de octubre de 2016 en Colombia, así como de discursos que produjeron agentes institucionales en el marco del debate en torno a la llamada “ideología de género”. Seguiré las propuestas metodológicas de Van Dijk, Lazar y de Kress y Van Leeuwen para lograr una aproximación desde el análisis crítico multimodal del discurso en clave feminista. El análisis muestra la capacidad performativa de discursos dirigidos al nivel micropolítico y biopolítico, pero con efectos materiales sobre las políticas macro de la vida y la muerte.

  10. FUTURE FOREIGN LANGUAGE TEACHERS' SOCIAL AND COGNITIVE COLLABORATION IN AN ONLINE ENVIRONMENT

    Directory of Open Access Journals (Sweden)

    Nike Arnold

    2006-01-01

    Full Text Available Discussion boards provide an interactive venue where new and future language teachers can reflect, evaluate, solve problems or simply exchange ideas (e.g., Bonk, Hansen, Grabner-Hagen, Lazar, & Mirabelli, 1996; DeWert, Babinski, & Jones, 2003; Kumari, 2001; Pawan, Paulus, Yalcin, & Chang, 2003. In addition, encouraging future teachers to learn with technology before teaching with it allows them to become comfortable using various computer applications. This article examines transcripts from a semester-long asynchronous discussion between foreign language methodology classes at two different universities. Social and cognitive presence in the discussions was analyzed using Garrison, Anderson, and Archer’s Framework of a Community of Inquiry (2001. The results indicate that students engaged in a high degree of interactivity as well as all types of social and cognitive presence. These findings indicate that students not only progressed in their cognitive understanding of the pedagogical topics, but also employed social presence, the more dominant of the two, to aid their discussions. The topics seemed to play an important role in the type of cognitive activity evident in the discussions. These results differ from those of studies which found that students did not engage in interactivity (Henri, 1995; Pena-Shaff & Nicholls, 2004 and others which noted low levels of social presence (Garrison, et al. 2001; Meyer, 2003.

  11. Canyons and gorges as potential geotourism destinations in Serbia: comparative analysis from two perspectives - general geotourists' and pure geotourists'

    Science.gov (United States)

    Božić, Sanja; Tomić, Nemanja

    2015-10-01

    Serbia represents one of those countries which have not yet differentiated themselves on the world tourism map. However, it has an immense but still unrevealed potential for geotourism development. In this paper we analyzed several remarkable canyons and gorges of great scientific importance and geotourism potential. These sites include the Djerdap Gorge and Lazar River Canyon, located in Eastern Serbia and the Ovcar-Kablar Gorge and Uvac Canyon located in Western Serbia. One of the main goals of this paper was to analyze and compare the current state and tourism potential of these geosites by using the M-GAM model for geosite assessment. However, the principal aim of the paper is to analyze how important is each subindicator in the assessment process for different market segments. In this paper, we also analyzed how giving different importance to subindicators can influence the position of the geosites in the matrix indicating different assessment done by two chosen market segments. The research showed that general geotourists appreciate considerably different values when assessing a geosite in comparison to pure geotourists. The paper can be used as framework for developing the tourism management strategy of geosites taking into consideration the needs and preferences of the target market segments.

  12. Zusammenfassungen in Deutsch

    Directory of Open Access Journals (Sweden)

    Nora Wiedenmann

    2010-04-01

    Full Text Available 1. Eine hybride Heuristik zur Lösung des Triangulations-ProblemsGloria Cerasela Crişan, Camelia Mihaela Pintea2. Vorschlag einer datenbasierten Architektur für eine Kardiologie-Netzwerk-AnwendungHorea Adrian Greblă, Călin Ovidiu Cenan3. Erziehungswissenschaftliche Forschung: zur technologischen Dimension des PrivatlebensLiliana Mâţă4. Ausgewählte Operationen für Assembler EncodingTomasz Praczyk5. Computerbasierte Programme zur Sprechtherapie bei Dyslalie und Dyslexie-DysgraphieIolanda Tobolcea, Mirela Danubianu6. Gedacht soap, jedoch gesagt ‘oaps’. Der Lautvorspann (Sound Preparation Period: Backward Calculation von der Äußerung bis zur Muskel-InnervierungNora Wiedenmann7. State of the Art: Lösungskonzepte für KoalitionsspieleSimina Brânzei8. Die Faktoren von Intelligenz-Entwicklung und individuelle PerformanzGheorghe Dumitriu9. Einige Ergebnisse zur Fuzzy-TheorieAngel Garrido10. Wissensfusion in akademischen NetzwerkenHorea Adrian Grebla, Calin Ovidiu Cenan, Liana Stanca11. Eine Delphi-Anwendung für die syntaktische und lexikalische Analyse eines Phrasen verwendenden Cocke-Younger-Kasami -Algorithmus.Bogdan Pătruţ, Ioana Boghian12. Über ein Problem von Passdaten bei Gebrauch von Bézier-KurvenCarmen Violeta Muraru13. State of the Art: Verifizierung von Unterschriften-Biometrie-DatenMohamed Soltane, Noureddine Doghmane, Noureddine Guersi14. Die Neurowissenschaften des Lehrens von Erzähltexten: zur Erleichterung der sozialenund emotionalen EntwicklungLisa Whalen15. Logische Grundlagen der Künstlichen IntelligenzAngel Garrido16. Psychologisch-mystische Aspekte zu St. Evagrius Ponticus und St. Maximus dem BekennerGheorghe Teofil Popovici17. Geo-spatiale Analyse-StrukturElisabeta Antonia Haller18. Henric Sanielevici – von der Literaturkritik zur Anatomie oder Wie beeinflusst der Schädelumfang die Literatur?Adrian Jicu19. Wie komplex das menschliche Gehirn funktioniert: die beiden HemisphärenIulia Cristina Timofti20

  13. Predictors of renal and patient outcomes in anti-GBM disease: clinicopathologic analysis of a two-centre cohort.

    Science.gov (United States)

    Alchi, Bassam; Griffiths, Meryl; Sivalingam, Murugan; Jayne, David; Farrington, Ken

    2015-05-01

    Patients with anti-glomerular basement membrane (GBM) disease are at increased risk of morbidity and mortality from renal failure, pulmonary haemorrhage or complications of treatment. One-third also have circulating anti-neutrophil cytoplasmic antibodies (ANCA). The aim of this study was to determine the clinicopathologic predictors of patient and renal outcomes in anti-GBM disease with or without ANCA. Retrospective review of 43 patients diagnosed with anti-GBM disease over 20 years in two centres, including nine with dual anti-GBM and ANCA positivity. Renal biopsies from 27 patients were scored for the presence of active and chronic lesions. Dual-positive patients were almost 20 years older than those with anti-GBM positivity alone (P = 0.003). The overall 1-year patient and renal survivals were 88 and 16%, respectively. Oligoanuria at diagnosis was the strongest predictor of mortality; none of the 16 patients without oligoanuria died. In a Cox regression model excluding oligoanuria, age was the only other independent predictor of survival. Pulmonary haemorrhage and dialysis dependence did not influence mortality. Thirty-five of the forty-three (81%) patients required dialysis at presentation, including all nine dual-positive patients. Of them, only two (5.7%) regained renal function at 1 year. By logistic regression, oligoanuria at diagnosis and percentage of crescents were independent predictors of dialysis independence at 3 months. However, in biopsied patients, the presence of crescents (>75%) added little to the presence of oligoanuria in predicting dialysis independence. Histological activity and chronicity indices did not predict renal outcome. Two of the nine (22%) dual-positive patients relapsed compared with none of the anti-GBM alone patients. Seven patients received kidney transplants without disease recurrence. Oligoanuria is the strongest predictor of patient and renal survival while percentage of glomerular crescents is the only pathologic

  14. Nonorgan-specific autoantibodies in HIV-infected patients in the HAART era.

    Science.gov (United States)

    Iordache, Laura; Bengoufa, Djaouida; Taulera, Olivier; Rami, Agathe; Lascoux-Combe, Caroline; Day, Nesrine; Parrinello, Maguy; Sellier, Pierre-Olivier; Molina, Jean-Michel; Mahr, Alfred

    2017-03-01

    Nonorgan-specific autoantibodies (AAbs) are used for diagnosing autoimmune diseases but can also be detected in other conditions. We carried out a cross-sectional study with the aim to screen HIV1-infected patients in the era of highly active antiretroviral therapy (HAART) for AAbs and to analyze the association of their presence with hypergammaglobulinemia and immunovirological status.Blood samples from HIV1-infected patients without major concomitant illnesses followed in 2 hospitals in Paris, France were tested for immunovirological status, serum immunoglobulin G (IgG) level, antinuclear antibodies (ANAs), anti-double-stranded DNA (anti-dsDNA), anti-extractable nuclear antigens (anti-ENAs), anticardiolipin (aCL), anti-β2glycoprotein1 (anti-β2GP1), and antineutrophil cytoplasmic antibodies (ANCAs). Clinically relevant AAbs were defined as ANAs with titers ≥1:160, anti-dsDNA or anti-ENA antibodies; aCL or anti-β2GP1 antibodies with a level ≥40 U/ml; and ANCAs reacting with proteinase 3 or myeloperoxidase.We included 92 patients (mean age 47 years, men 55%, sub-Saharan African background 55%, HAART 85%, mean CD4 lymphocyte count 611/mm, viral load < 40 copies/mL 74%). At least 1 AAb was detected in 45% of patients, mostly ANAs (33%) and ANCAs (13%); 12% had ≥1 clinically relevant AAb. Above-normal IgG levels were found in 71% of patients. We found an inverse association between the presence of ≥1 AAb and CD4 lymphocyte count (P = 0.03) and between above-normal IgG levels and duration of virological control (P = 0.02) and non-sub-Saharan African background (P = 0.001).In sum, in HIV1-infected patients without any major concomitant illness in the HAART era, the prevalence of AAbs remains high but AAb patterns leading to high suspicion of autoimmune diseases are rather uncommon. AAb presence is associated with reduced CD4 lymphocyte count but not hypergammaglobulinemia.

  15. [Cardiac involvement in Churg-Strauss syndrome].

    Science.gov (United States)

    Brucato, Antonio; Maestroni, Silvia; Masciocco, Gabriella; Ammirati, Enrico; Bonacina, Edgardo; Pedrotti, Patrizia

    2015-09-01

    Churg-Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis, characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring among patients with asthma and tissue eosinophilia. EGPA is classified as a small and medium-sized vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) and the hypereosinophilic syndrome. Typical clinical features include asthma, sinusitis, transient pulmonary infiltrates and neuropathy. Blood eosinophils are often >1500/µl or more than 10% on the differential leukocyte count. Blood eosinophils should always be tested in unexplained cardiac disorders, and may normalize even after low doses of corticosteroids. ANCA are positive in 40-60% of cases, mainly anti-myeloperoxidase. Heart involvement occurs in approximately 15-60% of EGPA patients, especially those who are ANCA negative. Any cardiac structure can be involved, and patients present with myocarditis, heart failure, pericarditis, arrhythmia, coronary arteritis, valvulopathy, intracavitary cardiac thrombosis. Although cardiovascular involvement is usually an early manifestation, it can also occur later in the course of the disease. A significant proportion of patients with cardiac involvement is asymptomatic. In the absence of symptoms and major ECG abnormalities, cardiac involvement may be detected in nearly 40% of the patients. All patients with EGPA should be studied not only with a detailed history of cardiac symptoms and ECG, but also with echocardiography; if abnormalities are detected, a cardiac magnetic resonance study should be performed. Coronary angiography and endomyocardial biopsy should be reserved to selected cases. Heart involvement carries a poor prognosis and causes 50% of the deaths of these patients. It is often insidious and underestimated. Optimal therapy is therefore important and based on high-dose corticosteroids plus immunosuppressive

  16. [Pulmonary Manifestations of Vasculitis].

    Science.gov (United States)

    von Vietinghoff, S

    2016-11-01

    The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation. Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides. Life threatening pulmonary hemorrhage and irreversible damage of other organs, frequently the kidney, are important complications necessitating rapid diagnosis of these conditions.Vasculitides are rare diseases of multiple organs and therapies including biologics are evolving rapidly, requiring cooperation of specialities and with specialized centres to achieve best patient care. All involved physicians should be aware of typical complications of immunosuppressive therapy. © Georg Thieme Verlag KG Stuttgart · New York.

  17. Established and emerging biological activity markers of inflammatory bowel disease

    DEFF Research Database (Denmark)

    Nielsen, O H; Vainer, B; Madsen, S M

    2000-01-01

    Assessment of disease activity in inflammatory bowel disease (IBD), i.e., ulcerative colitis (UC) and Crohn's disease (CD), is done using clinical parameters and various biological disease markers. Ideally, a disease marker must: be able to identify individuals at risk of a given disorder......, be disease specific, mirror the disease activity and, finally, be easily applicable for routine clinical purposes. However, no such disease markers have yet been identified for IBD. In this article, classical disease markers including erythrocyte sedimentation rate, acute phase proteins (especially...... orosomucoid and CRP), leukocyte and platelet counts, albumin, neopterin, and beta2-microglobulin will be reviewed together with emerging disease markers such as antibodies of the ANCA/ASCA type, cytokines (e.g., IL-1, IL-2Ralpha, IL-6, IL-8, TNF-alpha, and TNF-alpha receptors) and with various adhesion...

  18. Cryoglobulinemic vasculitis preceding diagnosis of Carney Complex

    DEFF Research Database (Denmark)

    Glerup, Mia; Stausbøl-Grøn, Brian; Heuck, Carsten

    2014-01-01

    Carney complex (CNC) is a disorder characterized by skin pigmentary abnormalities and benign cardiac, endocrine, skin and neuronal tumors. We present a previously healthy 12-year-old boy with recurrent pain and swelling of the feet. One year later he presented with stiffness of the fingers...... and the toes were painful with no swelling. Rheumatoid factor, ANCA and ANA were all negative but cryoglobulins were positive. Unusual lentigines were noted. The monthly attacks with pain and joint swelling were treated sufficiently with prednisolone and azathioprine. Two years later he was admitted....... The lentigines and myxoma led to the suggestion of Carney Complex and the diagnosis was confirmed by a PRKAR1A mutation found in the child as well as the mother....

  19. Differential expression of granulopoiesis related genes in neutrophil subsets distinguished by membrane expression of CD177

    DEFF Research Database (Denmark)

    Hu, Nan; Mora-Jensen, Helena; Theilgaard-Mønch, Kim

    2014-01-01

    OBJECTIVE: Differential gene expression in CD177+ and CD177- neutrophils was investigated, in order to detect possible differences in neutrophil function which could be related to the pathogenesis of ANCA-associated Vasculitides (AAV). METHODS: Neutrophils were isolated from healthy controls (HC......) with high, negative or bimodal CD177 expression, and sorted into CD177+ and CD177- subpopulations. Total RNA was screened for expression of 24,000 probes with Illumina Ref-8 Beadchips. Genes showing differential expression between CD177+ and CD177- subsets in microarray analysis were re-assessed using...... quantitative-PCR. CD177 expression on neutrophil precursors in bone marrow was analyzed using quantitative PCR and flowcytometry. RESULTS: The proportion of CD177+ cells increased during neutrophil maturation in bone marrow. Fold change analysis of gene expression profile of sorted CD177+ and CD177...

  20. L’Image à Byzance. Une nouvelle lecture des textes anciens

    Directory of Open Access Journals (Sweden)

    Maria Zoubouli

    2010-05-01

    Full Text Available Thèse soutenue le 9 avril 2009Composition du juryFrançois Hartog,André Guillou,Ennio Concina,Anca Vasiliu,Léon Pressouyre,(Pré-rapporteurs : Leslie Brubaker, Ennio Concina.RésuméÀ Byzance eikon désigne indistinctement toute forme de représentation et son usage, relève de multiples régimes, conceptuels aussi bien que pratiques. Nous partons des premiers discours apologétiques issus de la crise iconoclaste pour chercher en aval, dans la littérature antérieure, la théorie byzantine de l’image, ...

  1. Dense Deposit Disease Mimicking a Renal Small Vessel Vasculitis

    Science.gov (United States)

    Singh, Lavleen; Bhardwaj, Swati; Sinha, Aditi; Bagga, Arvind; Dinda, Amit

    2016-01-01

    Dense deposit disease is caused by fluid-phase dysregulation of the alternative complement pathway and frequently deviates from the classic membranoproliferative pattern of injury on light microscopy. Other patterns of injury described for dense deposit disease include mesangioproliferative, acute proliferative/exudative, and crescentic GN. Regardless of the histologic pattern, C3 glomerulopathy, which includes dense deposit disease and C3 GN, is defined by immunofluorescence intensity of C3c two or more orders of magnitude greater than any other immune reactant (on a 0–3 scale). Ultrastructural appearances distinguish dense deposit disease and C3 GN. Focal and segmental necrotizing glomerular lesions with crescents, mimicking a small vessel vasculitis such as ANCA-associated GN, are a very rare manifestation of dense deposit disease. We describe our experience with this unusual histologic presentation and distinct clinical course of dense deposit disease, discuss the pitfalls in diagnosis, examine differential diagnoses, and review the relevant literature. PMID:26361799

  2. Infarto agudo do miocárdio como apresentação inicial de granulomatose eosinofílica com poliangiite (anteriormente, síndrome de Churg Strauss

    Directory of Open Access Journals (Sweden)

    Wahinuddin Sulaiman

    2014-10-01

    Full Text Available A granulomatose eosinofílica com poliangiite é uma vasculite primária rara, caracterizada por hipereosinofilia, asma de surgimento tardio e granulomas eosinofílicos extravasculares. Relatamos um caso apresentado inicialmente com infarto do miocárdio e que, ulteriormente, teve prosseguimento apenas com asma, manifestações cutâneas e neuropatia periférica. Os parâmetros laboratoriais revelaram hipereosinofilia com um padrão perinuclear negativo de autoanticorpos citoplásmicos antineutrófilos (p-ANCA. A biópsia de pele demonstrou vasculite leucocitoclástica com infiltração eosinofílica, diante de uma angiografia coronária normal. Os sintomas do paciente melhoraram com metilprednisolona IV, pulsoterapia com ciclofosfamida e azatioprina.

  3. [Report on the 34th meeting of the German Clinical Immunology Workgroup, Frankfurt, 03.-04.11.2006].

    Science.gov (United States)

    Aries, P M; Witte, T; Lamprecht, P

    2007-02-01

    The annual meeting of the Clinical Immunology Workgroup focused on autoimmune vasculitides. The role of innate immunity, T- and B-cells, and innovative therapies for autoimmune vasculitides was discussed. Further topics of the meeting were the role of endothelial microparticles, ghrelin and leptin, regulatory and effector-memory T-cells in ANCA-associated vasculitides, as well as the lethal midline granuloma, intracytoplasmic cytokine-profile in Behcet's disease, autoantibodies in rheumatoid arthritis, polyarteritis nodosa with cranial manifestation, ILT6 as genetic marker in multiple sclerosis and Sjögren's syndrome, alpha-fodrin autoantibodies in multiple sclerosis, interferon-g autoantibodies in a patient with atypical mycobacteriosis, and autoreactive T-cells in murine lupus.

  4. Erasmus syndrome: Association of silicosis and systemic sclerosis

    Directory of Open Access Journals (Sweden)

    Reena K Sharma

    2018-01-01

    Full Text Available Silicosis is an inflammatory disease of the lung characterized by irreversible lung fibrosis which develops from prolonged pulmonary inhalation and retention of crystalline silica and immune reaction. It mainly appears as an occupational hazard in persons involved in stone-quarrying, mining, and sand blasting. Crystalline silica is not only known to be responsible for silicosis but also for other autoimmune diseases including systemic lupus erythematosus (SLE, rheumatoid arthritis (RA-Caplan syndrome, systemic sclerosis (SSc, and antineutrophil cytoplasmic antibody (ANCA-related vasculitis. Erasmus syndrome is the association of silica exposure and subsequent development of SSc. The limited numbers of cases reported in the literature were miners and only sporadically involved in other professionals. Here, we report a case of a 52 -year-old stone cutter who developed silicosis and SSc after 25 years of exposure.

  5. Levamisole-Contaminated Cocaine: An Emergent Cause of Vasculitis and Skin Necrosis

    Directory of Open Access Journals (Sweden)

    Osama Souied

    2014-01-01

    Full Text Available The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care.

  6. Combined Churg-Strauss syndrome and allergic bronchopulmonary aspergillosis - case report and review of the literature.

    Science.gov (United States)

    Ren, Shaohua

    2013-01-01

    A rare case of combined Churg-Strauss syndrome (CSS) and allergic bronchopulmonary aspergillosis (ABPA) was presented. A 41-year-old woman was diagnosed with CSS based upon asthma, eosinophilia (23%), chest radiographic findings, paranasal sinusitis, peripheral neuropathy and positive p- anti-neutrophil cytoplasmic antibodies (pANCA). The diagnosis of ABPA was established on the pathological findings of allegic mucin impaction and fungal hyphae on lung biopsy. It was further proved by positive serum IgE and IgG antibodies specific to afumigatus. The clinical investigation features were reviewed in the patients with combined CSS and ABPA. All patients had the time sequence of the development of CSS after ABPA uniformly, suggesting immunopathogenesis involving the emergence of CSS. The role of lung biopsy in the diagnosis of the condition was emphasized. © 2012 Blackwell Publishing Ltd.

  7. Idiopathic Pulmonary Hemosiderosis in a Young Adult Patient: A Rare Case

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    Abhishek Agarwal

    2018-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is often an ignored and rare cause of diffuse alveolar hemorrhage (DAH. It is characterized by triad of hemoptysis, anemia, and alveolar opacity on radiology. It is a diagnosis of exclusion, established after ruling out other causes of DAH such as Goodpasture’s syndrome, large vessel vasculitis, small vessel vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA (Wegener’s granulomatosis, Churg–Strauss syndrome, microscopic polyangiitis, immune complex-related vasculitis (collagen vascular diseases, Henoch–Schönlein purpura, mixed cryoglobulinemia drug reactions, anticoagulation and thrombocytopenia. Though it is a disease primarily affecting children, we hereby report a case of IPH in an adult patient who responded dramatically to oral corticosteroid.

  8. [Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

    Science.gov (United States)

    Guillevin, Loïc

    2012-10-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), is a rare necrotizing vasculitis of small-sized vessels, associated to antimyeloperoxydase ANCA in 40% of patients. EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present, prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present. Copyright © 2012. Published by Elsevier Masson SAS.

  9. Churg-Strauss syndrome and hemorragic vasculitis

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    Rui Moreira Marques

    2011-12-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the lefthand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome.

  10. Churg-Strauss syndrome and hemorrhagic vasculitis

    Science.gov (United States)

    Marques, Rui Moreira; Cabral, Ana Rita; Monteiro, Antonio; Henriques, Pedro

    2011-01-01

    Churg-Strauss syndrome (CSS) is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the left-hand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome. PMID:25386301

  11. Established and emerging biological activity markers of inflammatory bowel disease

    DEFF Research Database (Denmark)

    Nielsen, O H; Vainer, B; Madsen, S M

    2000-01-01

    orosomucoid and CRP), leukocyte and platelet counts, albumin, neopterin, and beta2-microglobulin will be reviewed together with emerging disease markers such as antibodies of the ANCA/ASCA type, cytokines (e.g., IL-1, IL-2Ralpha, IL-6, IL-8, TNF-alpha, and TNF-alpha receptors) and with various adhesion......Assessment of disease activity in inflammatory bowel disease (IBD), i.e., ulcerative colitis (UC) and Crohn's disease (CD), is done using clinical parameters and various biological disease markers. Ideally, a disease marker must: be able to identify individuals at risk of a given disorder......, be disease specific, mirror the disease activity and, finally, be easily applicable for routine clinical purposes. However, no such disease markers have yet been identified for IBD. In this article, classical disease markers including erythrocyte sedimentation rate, acute phase proteins (especially...

  12. Atypical Plasmacytic Proliferation in a Case of C3 Glomerulopathy

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    Osama Elfituri MD

    2017-02-01

    Full Text Available An 11-year-old Hispanic female underwent evaluation of asymptomatic proteinuria and hematuria. The patient denied fever, edema, and gross hematuria. Urinalysis showed mild proteinuria, and a urine microscopic examination revealed red blood cells. Screening tests for glomerulonephritis revealed a low C3 and negative ANA, ASO, DNAse-B, and ANCA. Histological examination of a renal biopsy specimen showed glomeruli with endocapillary proliferation, a predominant C3 deposition in the capillary loops by immunofluorescence, and electron dense deposits in the mesangium, paramesangium, and capillary walls by electron microscopy consistent with a diagnosis of C3 glomerulopathy. An interstitial plasmacytosis was also present with focal clustering of plasma cells, which were found to be kappa light chain restricted by in situ hybridization suggestive of a clonal proliferation. One can speculate that these plasma cells may be directly responsible for the renal pathology that was seen.

  13. Granulomatosis with polyangiitis mimicking infective endocarditis in an adolescent male.

    Science.gov (United States)

    Varnier, Giulia Camilla; Sebire, Neil; Christov, Georgi; Eleftheriou, Despina; Brogan, Paul A

    2016-09-01

    Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA. We provide an overview of the limited literature relating to cardiac involvement in GPA, and the diagnostic challenge relating to infective endocarditis in this context, especially focusing on the interpretation of the antineutrophil cytoplasmic antibody (ANCA) and the characteristic clinical features to identify in order to promptly recognise GPA, since timely diagnosis and treatment are essential for this potentially life-threatening condition.

  14. Propilthiouracil-induced diffuse pulmonary hemorrhage: a case report with the clinical and radiologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Young Jun; Kim, Joung Sook; Kim, Ji Young; Choi, Soo Jeon [Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of)

    2007-05-15

    Propylthiouracil (PTU) is a drug that's used to manage hyperthyroidism and it can, on rare occasions, induce antineutrophil cytoplasmic antibody-associated vasculitis that involved multiple organ systems and it can also cause extremely rare isolated or diffuse pulmonary hemorrhage. We report here on a case of a patient who develop diffuse pulmonary hemorrhage after she had been taking PTU for five years. The patient is a 33-year-old woman who presented with hemoptysis. Simple chest radiographs and the chest CT showed bilateral ground-glass opacity, consolidation and pulmonary arterial hypertension. The bronchoalveolar lavage fluid revealed alveolar hemorrhage. The laboratory values showed increased perinuclear-antineutrophil cytoplasmic antibody ({rho} - ANCA) and anti-peroxidase antibody titers.

  15. Granulomatosis with polyangiitis presenting as facial nerve palsy in a teenager.

    Science.gov (United States)

    Wang, James C; Leader, Brittany A; Crane, Ryan A; Koch, Bernadette L; Smith, Matthew M; Ishman, Stacey L

    2018-04-01

    Granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis) is an autoimmune systemic small-vessel vasculitis, associated with the presence of anti-neurophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA). It is characterized by necrotizing granulomas, usually affecting the airways and kidneys. GPA should be considered when patients do not improve despite adequate treatment of otologic symptoms, when patients have unspecific symptoms suggesting systemic disease (e.g. fever, malaise), or when other organs are involved (kidney, lungs, etc.). We present an interesting case of a 14-year-old female with eight-weeks of bilateral otalgia, unilateral facial nerve palsy, decreased appetite, and fatigue refractory to steroid, anti-viral, and antibiotic treatment ultimately diagnosed with GPA. Copyright © 2018. Published by Elsevier B.V.

  16. Diagnostic Consideration for Sinonasal Wegener’s Granulomatosis Clinically Mistaken for Carcinoma

    Directory of Open Access Journals (Sweden)

    Cristina La Rosa

    2013-01-01

    Full Text Available We report a case of Wegener’s granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography. However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener’s granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener’s granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener’s granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes.

  17. Diagnostic Consideration for Sinonasal Wegener's Granulomatosis Clinically Mistaken for Carcinoma

    Science.gov (United States)

    La Rosa, Cristina; Emmanuele, Carmela; Tranchina, Maria Grazia; Ippolito, Massimo; Cosentino, Sebastiano; Saita, Vincenzo; Fraggetta, Filippo

    2013-01-01

    We report a case of Wegener's granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography). However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener's granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener's granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener's granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes. PMID:24106630

  18. Wegener's granulomatosis occurring de novo during pregnancy.

    Science.gov (United States)

    Alfhaily, F; Watts, R; Leather, A

    2009-01-01

    Wegener's granulomatosis (WG) is rarely diagnosed during the reproductive years and uncommonly manifests for the first time during pregnancy. We report a case of de novo WG presenting at 30 weeks gestation with classical symptoms of WG (ENT, pulmonary). The diagnosis was confirmed by radiological, laboratory, and histological investigations. With a multidisciplinary approach, she had a successful vaginal delivery of a healthy baby. She was treated successfully by a combination of steroids, azathioprine and intravenous immunoglobulin in the active phase of disease for induction of remission and by azathioprine and steroids for maintenance of remission. The significant improvement in her symptoms allowed us to continue her pregnancy to 37 weeks when delivery was electively induced. Transplacental transmission of PR3-ANCA occurred but the neonate remained well. This case of de novo WG during pregnancy highlights the seriousness of this disease and the challenge in management of such patients.

  19. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis.

    Science.gov (United States)

    Veronese, F V; Dode, R S O; Friderichs, M; Thomé, G G; da Silva, D R; Schaefer, P G; Sebben, V C; Nicolella, A R; Barros, E J G

    2016-01-01

    Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs) and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320), as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  20. Vasculitis inducida por metimazol: Reporte de caso

    Directory of Open Access Journals (Sweden)

    Miguel Pinto

    2011-07-01

    Full Text Available Se reporta el caso de una paciente con enfermedad de Graves, que presentó vasculitis asociada al uso de metimazol. Mujer de 14 años, que acudió a consulta por presentar intolerancia al calor, tremor distal y palpitaciones. El examen físico mostró bocio difuso, y el perfil tiroideo, TSH suprimida y hormonas tiroideas elevadas. Los anticuerpos antiperoxidasa tiroidea fueron positivos. Se inició tratamiento con metimazol y beta bloqueadores. Después de 20 días, la paciente regresó por presentar malestar general, fiebre, poliartralgia, lesiones cutáneas maculopapulares y edema de miembros inferiores. Los anticuerpos antinucleares fueron negativos y los anticuerpos anticitoplasma de los neutrófilos (ANCA, positivos. Se suspendió el metimazol y se inició prednisona. Después de 10 días de tratamiento, las molestias desaparecieron y la paciente recibió I 131.Las vasculitis asociadas al uso de tionamidas son poco frecuentes, no dependen de la dosis y están asociadas a la presencia de anticuerpos tipo ANCA. Clásicamente, afectan a los vasos pequeños de la piel; sin embargo, también pueden afectar los riñones y pulmones. El cuadro clínico se caracteriza por artralgias y mialgias. En algunos casos puede ocurrir insuficiencia renal de grado variable. En la mayoría de casos, el cuadro remite con la suspensión de la droga; pero, en algunos se requiere el uso de glucocorticoides o inmunosupresores.(Rev Med Hered 2011;22:147-150.

  1. Diagnosis and classification of Goodpasture's disease (anti-GBM).

    Science.gov (United States)

    Hellmark, Thomas; Segelmark, Mårten

    2014-01-01

    Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies that bind to the basement membranes and activate the classical pathway of the complement system, which start a neutrophil dependent inflammation. The diagnosis of anti-GBM disease relies on the detection of anti-GBM antibodies in conjunction with glomerulonephritis and/or alveolitis. Overt clinical symptoms are most prominent in the glomeruli where the inflammation usually results in a severe rapidly progressive glomerulonephritis. Despite modern treatment less than one third of the patients survive with a preserved kidney function after 6 months follow-up. Frequencies vary from 0.5 to 1 cases per million inhabitants per year and there is a strong genetic linkage to HLA-DRB1(∗)1501 and DRB1(∗)1502. Essentially, anti-GBM disease is now a preferred term for what was earlier called Goodpasture's syndrome or Goodpasture's disease; anti-GBM disease is now classified as small vessel vasculitis caused by in situ immune complex formation; the diagnosis relies on the detection of anti-GBM in tissues or circulation in conjunction with alveolar or glomerular disease; therapy is effective only when detected at an early stage, making a high degree of awareness necessary to find these rare cases; 20-35% have anti-GBM and MPO-ANCA simultaneously, which necessitates testing for anti-GBM whenever acute test for ANCA is ordered in patients with renal disease. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. Cocaine/levamisole-induced systemic vasculitis with retiform purpura and pauci-immune glomerulonephritis

    Directory of Open Access Journals (Sweden)

    F.V. Veronese

    2016-01-01

    Full Text Available Levamisole has been increasingly used as an adulterant of cocaine in recent years, emerging as a public health challenge worldwide. Levamisole-associated toxicity manifests clinically as a systemic vasculitis, consisting of cutaneous, hematological, and renal lesions, among others. Purpura retiform, cutaneous necrosis, intravascular thrombosis, neutropenia, and less commonly crescentic nephritis have been described in association with anti-neutrophil cytoplasmic antibodies (ANCAs and other autoantibodies. Here we report the case of a 49-year-old male who was a chronic cocaine user, and who presented spontaneous weight loss, arthralgia, and 3 weeks before admission purpuric skin lesions in the earlobes and in the anterior thighs. His laboratory tests on admission showed serum creatinine of 4.56 mg/dL, white blood count 3,800/μL, hemoglobin 7.3 g/dL, urinalysis with 51 white blood cells/μL and 960 red blood cells/μL, and urine protein-to-creatinine ratio 1.20. Serum ANCA testing was positive (>1:320, as well as serum anti-myeloperoxidase and anti-proteinase 3 antibodies. Urine toxicology screen was positive for cocaine and levamisole, with 62.8% of cocaine, 32.2% of levamisole, and 5% of an unidentified substance. Skin and renal biopsies were diagnostic for leukocytoclastic vasculitis and pauci-immune crescentic glomerulonephritis, respectively. The patient showed a good clinical response to cocaine abstinence, and use of corticosteroids and intravenous cyclophosphamide. Last serum creatinine was 1.97 mg/dL, white blood cell count 7,420/μL, and hemoglobin level 10.8 g/dL. In levamisole-induced systemic vasculitis, the early institution of cocaine abstinence, concomitant with the use of immunosuppressive drugs in severe cases, may prevent permanent end organ damage and associate with better clinical outcomes.

  3. Carbon nanotubes toxicology and effects on metabolism and immunological modification in vitro and in vivo

    International Nuclear Information System (INIS)

    Chiaretti, M; Mazzanti, G; Mastrangelo, S; Di Sotto, A; Bosco, S; Porta, N; Deriu, G; Bellucci, S; Balasubramanian, C; De Bellis, G; Micciulla, F; Tiberia, A; Cucina, A; Le Foche, F; Carru, G A; Masciangelo, R; Chiaretti, A M

    2008-01-01

    The aim of this research is focused on the biological effects of multi wall carbon nanotubes (MWCNTs) on three different human cell types, laboratory animals in vivo, and immunological effects. Large numbers of researchers are directly involved in the handling of nanostructured materials such as MWCNTs and nanoparticles. It is important to assess the potential health risks related to their daily exposure to carbon nanotubes. The administration of sterilized nanosamples has been performed on laboratory animals, in both acute and chronic administration, and the pathological effects on the parenchymal tissues have been investigated. We studied the serum immunological modifications after intraperitoneal administration of the MWCNTs. We did not observe any antigenic reaction; the screening of ANA, anti-ENA, anti-cardiolipin, C-ANCA and P-ANCA was negative. No quantitative modification of immunoglobulins was observed, hence no modification of humoral immunity was documented. We also studied the effects of MWCNTs on the proliferation of three different cell types. MCF-7 showed a significant inhibition of proliferation for all conditions studied, whereas hSMCs demonstrated a reduction of cell growth only for the highest MWCNTs concentrations after 72 h. Also, no growth modification was observed in the Caco-2 cell line. We observed that a low quantity of MWCNTs does not provoke any inflammatory reaction. However, for future medical applications, it is important to realize prosthesis based on MWCNTs, through studying the corresponding implantation effects. Moreover, it has to be emphasized that this investigation does not address, at the moment, the carcinogenicity of MWCNTs, which requires a detailed follow-up investigation on the specific topic. In view of the subsequent and more extensive use of MWCNTs, especially in applications where carbon nanotubes are injected into the human body for drug delivery, as a contrast agent carrying entities for MRI, or as the basic

  4. VASCULITIS DE CHURG-STRAUSS: PRESENTACIÓN CLÍNICA COMO GLOMERULONEFRITIS EXTRACAPILAR NECROTIZANTE PAUCI-INMUNE CON NEFRITIS TUBULO-INTERSTICIAL EOSINOFÍLICA.

    Directory of Open Access Journals (Sweden)

    Fernanda da Cunha

    2004-01-01

    Full Text Available RESUMEN: Se presenta el caso clínico de una mujer de 81 años con antecedentes patología pulmonar obstructiva crónica "idiopática" que desarrolló un cuadro de insuficiencia renal aguda aparentemente prerrenal, con posterior oliguria y eosinofilia simulando una nefropatía túbulo-intersticial alérgica. La evolución atípica y la presencia de p-ANCA, sugerían una vasculitis, patología que se confirmó con la biopsia renal. Los resultados anatomopatológicos revelaron la existencia de una glomerulonefritis necrotizante con semilunas e infiltrado eosinófilo. Estos datos y el historial de la paciente llevaron al diagnóstico de vasculitis de Churg-Strauss. El tratamiento inicial con corticoides y ciclofosfamida y posteriormente con Azatioprina mostró excelentes resultados con mejoría de la función renal, de los parámetros inflamatorios y de la clínica sistémica, que la paciente mantuvo posteriormente. ABSTRACT: We report a case of a 81 year-old woman with idiopathic chronic obstructive pulmonary disease, who developed a functional acute renal failure with delayed oliguria and eosinophily, simulating an acute interstitial nephropathy. The unusual clinical course and the presence of antimyeloperoxidase antibodies (p-ANCA suggested a vasculitis; the renal biopsy confirmed this diagnosis. The histology revealed a crescentic glomerulonephritis with eosinophilic infiltration. With these data and the pulmonary history of the patient, a diagnosis of Churg-Struss Vasculitis was made. The initial treatment with steroids and ciclophosphamide switched later to azatioprine, was succeeded with a sustained improvement in renal function, inflammatory markers and clinical course.

  5. Effect of delayed diagnosis on disease course and management of Churg-Strauss syndrome: a retrospective study.

    Science.gov (United States)

    Sokołowska, Barbara; Szczeklik, Wojciech; Mastalerz, Lucyna; Kuczia, Paweł; Wodkowski, Michał; Stodółkiewicz, Edyta; Macioł, Karolina; Musiał, Jacek

    2013-03-01

    Delayed diagnosis in patients with Churg-Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease's initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p < 0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p < 0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p < 0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p < 0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.

  6. Childhood-onset eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome): a contemporary single-center cohort.

    Science.gov (United States)

    Gendelman, Samantha; Zeft, Andrew; Spalding, Steven J

    2013-06-01

    To date only 38 cases of childhood-onset eosinophilic granulomatosis with polyangiitis (cEGPA; formerly Churg-Strauss syndrome) have been reported. Additional patients with cEGPA could enhance the understanding of this rare and life-threatening condition. Our objectives were (1) to determine the frequency of specific organ system involvement; (2) to examine initial therapeutic regimen; and (3) to document disease and therapy-related morbidity in a contemporary cohort of patients with cEGPA. Retrospective review of patients evaluated at the Cleveland Clinic between 2003 and 2011 who met either American College of Rheumatology or Lanham criteria for EGPA and whose age was < 18 years at symptom onset. Nine patients (8 female; 7 white) were identified. Median age at onset of rhinitis/asthma symptom was 13 years and median age at diagnosis of cEGPA was 15 years. All patients demonstrated eosinophilia, upper airway disease (allergic rhinitis, chronic sinusitis, and/or nasal polyps), and pulmonary involvement. Other frequently involved organ systems included musculoskeletal (67%), gastrointestinal (67%), cutaneous (67%), neurologic (56%), and cardiac (44%). Antineutrophil cytoplasmic antibody (ANCA) serologies were negative in all patients. The medications used most frequently for initial therapy included oral (44%) or intravenous corticosteroids (56%) and azathioprine (67%). Disease or therapeutic complications occurred in half of the cohort and included heart failure, stroke, and sequela from longterm, high-dose steroids. Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. ANCA titers are often negative. Steroids are the mainstay of initial therapy but steroid-related side effects occur regularly.

  7. Carbon nanotubes toxicology and effects on metabolism and immunological modification in vitro and in vivo

    Science.gov (United States)

    Chiaretti, M.; Mazzanti, G.; Bosco, S.; Bellucci, S.; Cucina, A.; LeFoche, F.; Carru, G. A.; Mastrangelo, S.; Di Sotto, A.; Masciangelo, R.; Chiaretti, A. M.; Balasubramanian, C.; DeBellis, G.; Micciulla, F.; Porta, N.; Deriu, G.; Tiberia, A.

    2008-11-01

    The aim of this research is focused on the biological effects of multi wall carbon nanotubes (MWCNTs) on three different human cell types, laboratory animals in vivo, and immunological effects. Large numbers of researchers are directly involved in the handling of nanostructured materials such as MWCNTs and nanoparticles. It is important to assess the potential health risks related to their daily exposure to carbon nanotubes. The administration of sterilized nanosamples has been performed on laboratory animals, in both acute and chronic administration, and the pathological effects on the parenchymal tissues have been investigated. We studied the serum immunological modifications after intraperitoneal administration of the MWCNTs. We did not observe any antigenic reaction; the screening of ANA, anti-ENA, anti-cardiolipin, C-ANCA and P-ANCA was negative. No quantitative modification of immunoglobulins was observed, hence no modification of humoral immunity was documented. We also studied the effects of MWCNTs on the proliferation of three different cell types. MCF-7 showed a significant inhibition of proliferation for all conditions studied, whereas hSMCs demonstrated a reduction of cell growth only for the highest MWCNTs concentrations after 72 h. Also, no growth modification was observed in the Caco-2 cell line. We observed that a low quantity of MWCNTs does not provoke any inflammatory reaction. However, for future medical applications, it is important to realize prosthesis based on MWCNTs, through studying the corresponding implantation effects. Moreover, it has to be emphasized that this investigation does not address, at the moment, the carcinogenicity of MWCNTs, which requires a detailed follow-up investigation on the specific topic. In view of the subsequent and more extensive use of MWCNTs, especially in applications where carbon nanotubes are injected into the human body for drug delivery, as a contrast agent carrying entities for MRI, or as the basic

  8. CD45RC isoform expression identifies functionally distinct T cell subsets differentially distributed between healthy individuals and AAV patients.

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    Laurence Ordonez

    Full Text Available In animal models of anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis (AAV, the proportion of CD45RC T cell subsets is important for disease susceptibility. Their human counterparts are, however, functionally ill defined. In this report, we studied their distribution in healthy controls (HC, AAV patients and in Systemic lupus erythematous (SLE patients as disease controls. We showed that CD45RC expression level on human CD4 and CD8 T cells identifies subsets that are highly variable among individuals. Interestingly, AAV patients exhibit an increased proportion of CD45RC(low CD4 T cells as compared to HC and SLE patients. This increase is stable over time and independent of AAV subtype, ANCA specificity, disease duration, or number of relapses. We also analyzed the cytokine profile of purified CD4 and CD8 CD45RC T cell subsets from HC, after stimulation with anti-CD3 and anti-CD28 mAbs. The CD45RC subsets exhibit different cytokine profiles. Type-1 cytokines (IL-2, IFN-gamma and TNF-alpha were produced by all CD45RC T cell subsets, while the production of IL-17, type-2 (IL-4, IL-5 and regulatory (IL-10 cytokines was restricted to the CD45RC(low subset. In conclusion, we have shown that CD45RC expression divides human T cells in functionally distinct subsets that are imbalanced in AAV. Since this imbalance is stable over time and independent of several disease parameters, we hypothesize that this is a pre-existing immune abnormality involved in the etiology of AAV.

  9. Clinical significance of autoantibodies in autoimmune hepatitis.

    Science.gov (United States)

    Liberal, Rodrigo; Mieli-Vergani, Giorgina; Vergani, Diego

    2013-10-01

    The accurate diagnosis and classification of autoimmune hepatitis (AIH) rely upon the detection of characteristic autoantibodies. Positivity for anti-nuclear (ANA) and/or anti-smooth muscle (SMA) autoantibodies defines AIH type 1 (AIH-1), whereas anti-liver kidney microsomal type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) define AIH type 2 (AIH-2). ANA and SMA, and less commonly anti-LKM1, have also been detected in de-novo autoimmune hepatitis developing after liver transplantation, a condition that may affect patients transplanted for non-autoimmune liver disease. The diagnostic autoantibodies associated with AIH-1 are also detected in the paediatric AIH/sclerosing cholangitis overlap syndrome, referred to as autoimmune sclerosing cholangitis (ASC). ASC, like adult primary sclerosing cholangitis, is often associated with atypical perinuclear anti-neutrophil cytoplasmic autoantibodies (p-ANCA), although p-ANCA are also detected in other autoimmune liver diseases. These associations highlight the necessity for simple and prompt diagnostic autoantibody testing, and the requirement for the accurate interpretation of the results of the tests in the clinical context. Fine-mapping of antigenic autoantibody targets has facilitated the development of rapid molecular assays that have the potential to revolutionise the field if properly standardised and when used in combination with classical immunofluorescence. Despite their diagnostic significance, the pathogenic role of the various autoantibodies and the mechanisms by which they can potentially inflict damage onto the liver cell remain a topic for further research. Copyright © 2013 Elsevier Ltd. All rights reserved.

  10. Personalized reminiscence therapy M-health application for patients living with dementia: Innovating using open source code repository.

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    Zhang, Melvyn W B; Ho, Roger C M

    2017-01-01

    Dementia is known to be an illness which brings forth marked disability amongst the elderly individuals. At times, patients living with dementia do also experience non-cognitive symptoms, and these symptoms include that of hallucinations, delusional beliefs as well as emotional liability, sexualized behaviours and aggression. According to the National Institute of Clinical Excellence (NICE) guidelines, non-pharmacological techniques are typically the first-line option prior to the consideration of adjuvant pharmacological options. Reminiscence and music therapy are thus viable options. Lazar et al. [3] previously performed a systematic review with regards to the utilization of technology to delivery reminiscence based therapy to individuals who are living with dementia and has highlighted that technology does have benefits in the delivery of reminiscence therapy. However, to date, there has been a paucity of M-health innovations in this area. In addition, most of the current innovations are not personalized for each of the person living with Dementia. Prior research has highlighted the utility for open source repository in bioinformatics study. The authors hoped to explain how they managed to tap upon and make use of open source repository in the development of a personalized M-health reminiscence therapy innovation for patients living with dementia. The availability of open source code repository has changed the way healthcare professionals and developers develop smartphone applications today. Conventionally, a long iterative process is needed in the development of native application, mainly because of the need for native programming and coding, especially so if the application needs to have interactive features or features that could be personalized. Such repository enables the rapid and cost effective development of application. Moreover, developers are also able to further innovate, as less time is spend in the iterative process.

  11. Schiff Bases of Benzothiazol-2-ylamine and Thiazolo[5,4-b] pyridin-2-ylamine as Anticonvulsants: Synthesis, Characterization and Toxicity Profiling.

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    Shukla, Rashmi; Singh, Ajeet P; Sonar, Pankaj K; Mishra, Mudita; Saraf, Shailendra K

    2016-01-01

    Schiff bases have a broad spectrum of biological activities like antiinflammatory, analgesic, antimicrobial, anticonvulsant, antitubercular, anticancer, antioxidant, anthelmintic and so forth. Thus, after a thorough perusal of literature, it was decided to conjugate benzothiazol-2-ylamine/thiazolo [5, 4-b] pyridin-2-ylamine with aromatic and heteroaromatic aldehydes to get a series of Schiff bases. Synthesis, characterization, in-silico toxicity profiling and anticonvulsant activity of the Schiff bases of Benzothiazol-2-ylamine and Thiazolo [5, 4-b] pyridin-2-ylamine. Aniline/4-aminopyridine was converted to the corresponding thiourea derivatives, which were cyclized to obtain benzothiazol-2-ylamine/thiazolo [5, 4-b] pyridin-2-ylamine. Finally, these were condensed with various aromatic and heteroaromatic aldehydes to obtain Schiff bases of benzothiazol-2-ylamine and thiazolo [5, 4-b] pyridin-2-ylamine. The synthesized compounds were characterized and screened for their anticonvulsant activity using maximal electroshock (MES) test and isoniazid (INH) induced convulsions test. In-silico toxicity profiling of all the synthesized compounds was done through "Lazar" and "Osiris" properties explorer. Majority of the compounds were more potent against MES induced convulsions than INH induced convulsions. Schiff bases of benzothiazol-2-ylamine were more effective than thiazolo [5, 4-b] pyridin-2-ylamine against MES induced convulsions. The compound benzothiazol-2-yl-(1H-indol-2-ylmethylene)-amine (VI) was the most potent member of the series against both types of convulsions. Compound VI exhibited the most significant activity profile in both the models. The compounds did not exhibit any carcinogenicity or acute toxicity in the in-silico studies. Thus, it may be concluded that the Schiff bases of benzothiazol-2-ylamine exhibit the potential to be promising and non-toxic anticonvulsant agents.

  12. Apology in cases of medical error disclosure: Thoughts based on a preliminary study.

    Science.gov (United States)

    Dahan, Sonia; Ducard, Dominique; Caeymaex, Laurence

    2017-01-01

    Disclosing medical errors is considered necessary by patients, ethicists, and health care professionals. Literature insists on the framing of this disclosure and describes the apology as appropriate and necessary. However, this policy seems difficult to put into practice. Few works have explored the function and meaning of the apology. The aim of this study was to explore the role ascribed to apology in communication between healthcare professionals and patients when disclosing a medical error, and to discuss these findings using a linguistic and philosophical perspective. Qualitative exploratory study, based on face-to-face semi-structured interviews, with seven physicians in a neonatal unit in France. Discourse analysis. Four themes emerged. Difference between apology in everyday life and in the medical encounter; place of the apology in the process of disclosure together with explanations, regrets, empathy and ways to avoid repeating the error; effects of the apology were to allow the patient-physician relationship undermined by the error, to be maintained, responsibility to be accepted, the first steps towards forgiveness to be taken, and a less hierarchical doctor-patient relationship to be created; ways of expressing apology ("I am sorry") reflected regrets and empathy more than an explicit apology. This study highlights how the act of apology can be seen as a "language act" as described by philosophers Austin and Searle, and how it functions as a technique for making amends following a wrongdoing and as an action undertaken in order that neither party should lose face, thus echoing the sociologist Goffmann's interaction theory. This interpretation also accords with the views of Lazare, for whom the function of apology is a restoration of dignity after the humiliation of the error. This approach to the apology illustrates how meaning and impact of real-life language acts can be clarified by philosophical and sociological ideas.

  13. Pragmatic Difficulties in the Production of the Speech Act of Apology by Iraqi EFL Learners

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    Mehdi Falih Al-Ghazalli

    2014-12-01

    Full Text Available The purpose of this paper is to investigate the pragmatic difficulties encountered by Iraqi EFL university students in producing the speech act of apology. Although the act of apology is easy to recognize or use by native speakers of English, non-native speakers generally encounter difficulties in discriminating one speech act from another. The problem can be attributed to two factors: pragma-linguistic and socio-pragmatic knowledge. The aim of this study is(1to evaluate the socio-pragmatic level of interpreting apologies as understood and used by Iraqi EFL university learners, (2 find out the level of difficulty they experience in producing apologies and(3 detect the reasons behind such misinterpretations and misuses. It is hypothesized that the socio-pragmatic interpretation of apology tends to play a crucial role in comprehending what is intended by the speaker. However, cultural gaps can be the main reason behind the EFL learners' inaccurate production of the act of apology. To verify the aforementioned hypotheses, a test has been constructed and administered to a sample of 70 fourth-year Iraqi EFL university learners, morning classes. The subjects' responses have been collected and linguistically analyzed in the light of an eclectic model based on Deutschmann (2003 and Lazare (2004. It has been concluded that the misinterpretation or difficulty Iraqi EFL students have faced is mainly attributed to their lack of socio-pragmatic knowledge. The interference of the learnersʹ first language culture has led to non-native productions of speech act of apology.

  14. Serbian translation of French Code of Civil Procedure from 1837: Part two: Legal terminology of the translation

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    Stanković Uroš N.

    2015-01-01

    Full Text Available The article deals with legal terms appearing in Serbian translation of French Code of Civil Procedure (Code de procédure civile, 1806 authored by Serbian writer and politician Lazar Zuban (1795-1850. The author made an attempt to determine whether the terms used by Zuban had existed in historical sources previous to the translator's work. If so, it would mean that Zuban was using already existing technical terms. In cases in which he failed to find certain legal term in texts older than Zuban's work, the author tried to establish if the unfound term had been the translator's invention. As to the terms of civil law, Zuban mostly took over words already present in Serbian vocabulary at the time. This fact is easily explainable: family, property, contracts, torts, inheritage are very present in people's everyday life, which brought about terminology of civil law to be relatively developed. On the contrary, terms belonging to the civil procedure were scarce because judiciary and court procedure in the time of Zuban's work were still being on rudimentary level. That is the reason why the translator had to forge his own legal terms. Zuban did not translate German legal terms (the translator used German translation of a Code as protograph mechanically; he was making effort to fathom the meaning of a word in question and find its adequate Serbian equivalent. In some cases that effort was fruitful. Nevertheless, in a long term Zuban's labor was in vain, as none of his forged words survived in Serbian legal terminology.

  15. The first marriage of despot Leonardo III Tocco

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    Zečević Nada

    2006-01-01

    Full Text Available The marriage between Leonardo III — the last member of the Tocco family who ruled the Heptanese (1448-1479 — and Milica Branković, the daughter of the Serbian Despot Lazar Branković and Helen Palaiologos, concluded in Dubrovnik on May 1 1463, is an intriguing issue: it was concluded under unusual circumstances, and its significance was variously presented by the couple's earlier and later contemporaries. An analysis of several historical sources (mainly those of documentary character, also some of a narrative nature shows that, despite the belittlement of its significance made by some of the pair's contemporaries, the marriage was generally seen as a prospective alliance, designed not only to satisfy the existential needs of the couple but also to enhance the interests of various important political factors of the time: Thomas Palaiologos, the authorities of Dubrovnik, the Roman Curia and Cardinal Bessarion. As widely known, the political prospectives opened by this marriage were not fully accomplished due to the short duration of this relationship (Milica died soon after the wedding. Yet this conjugal alliance proved useful for the Tocco party even after its conclusion. In the time following Leonardo's flight from the Ottomans to Naples (after 1479, he referred to the lineage with Milica as an alliance with the Byzantine imperial family, supposedly in order to achieve certain benefices from the Italian environment where he repatriated. In parallel to the analysis of the sources about the motives and significance of the first marriage of the last Tocco despot, in this paper I also deal with several pro-sopographic and topographic details of regional importance, mentioned on the occasion of the Tocco-Brankovic wedding ceremony (e.g. Bishop of Bologna Blasius Constantii Paliki, etc. .

  16. Moses as a role model in the Serbia charters after 1371 changing patterns

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    Vujošević Žarko

    2008-01-01

    Full Text Available The aspects of the Old Testament figure of Moses highlighted in the charters of post-Nemanjić Serbia, or under the Lazarević and Branković dynasties (1371-1459, testify to a changed attitude towards Old Testament role models. While members of the Nemanjić house such as the archbishop Sava I and the rulers Stefan of Dečani and Dušan look up to Moses as a "religious leader", a prayerful intercessor before God and a victorious warrior, all of that for the sake of the "chosen" people, the role he is assigned in the arengae of the charters issued by prince Lazar and despots Stefan Lazarević and Đurađ Branković is completely different. In the universal Christian context of the post-1371 arengae Moses figures as a "prophet" and the builder of the Tabernacle - a prefiguration of the Church, thereby epitomizing a major stage in the salvation history of humankind. The role of Moses, as well as that of David, the only other Old Testament figure still referred to in the charters of the period, has a universal ecclesiologically interpreted, significance. This new pattern of interpreting Moses implies that the ruler’s main virtue now becomes his concern for the "true faith" and the houses of God. The practice of the Nemanjićs as regards selection and interpretation of Old Testament themes is reestablished by the titular despots of the Branković dynasty. In their charters, the first part of the Bible with Moses as a popular leader reassumes a "national" character and becomes part of the ideological apparatus intended to posit the Serbs as a "New Israel".

  17. Origanum vulgare mediated green synthesis of biocompatible gold nanoparticles simultaneously possessing plasmonic, antioxidant and antimicrobial properties

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    Benedec D

    2018-02-01

    Full Text Available Daniela Benedec,1,* Ilioara Oniga,1,* Flavia Cuibus,1 Bogdan Sevastre,2 Gabriela Stiufiuc,3 Mihaela Duma,4 Daniela Hanganu,1 Cristian Iacovita,1 Rares Stiufiuc,1,5 Constantin Mihai Lucaciu1 1Faculty of Pharmacy, “Iuliu Haţieganu” University of Medicine and Pharmacy, 2Faculty of Veterinary Medicine, University of Agricultural Sciences and Veterinary Medicine, 3Faculty of Physics, “Babeş Bolyai” University, 4State Veterinary Laboratory for Animal Health and Safety, 5Department of Bionanoscopy, MedFuture Research Center for Advance Medicine, “Iuliu Haţieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania *These authors contributed equally to this work Purpose: The leaves and flowering stem of Origanum vulgare contain essential oils, flavonoids, phenolic acids and anthocyanins. We propose a new, simple, one-pot, O. vulgare extract (OVE mediated green synthesis method of biocompatible gold nanoparticles (AuNPs possessing improved antioxidant, antimicrobial and plasmonic properties.Materials and methods: Different concentrations of OVEs were used to reduce gold ions and to synthetize biocompatible spherical AuNPs. Their morphology and physical properties have been investigated by means of transmission electron microscopy, ultraviolet–visible absorption spectroscopy, photon correlation spectroscopy and Fourier transform infrared spectroscopy, whereas their plasmonic properties have been tested using surface-enhanced Raman spectroscopy (SERS. The antioxidant properties of nanoparticles (NPs have been evaluated by 2,2-diphenyl-1-picrylhydrazyl radical scavenging assay, and the antimicrobial tests were performed using the disk diffusion assay. Their cytotoxicity has been assessed by means of 3-(4,5-dimethylthiazol-2-yl-2,5-diphenyltetrazolium bromide assay.Results: The experimental results confirmed the successful synthesis of biocompatible, spherical, plasmonic NPs having a mean diameter of ~40 nm and an outstanding aqueous

  18. GENERAL ASPECTS OF BODY MEASURES, WEIGHT AND SCORE CONDITION FEMALE NELORE BREED (Bos taurus indicus ON THE PERIOD OF 12 MONTHS ESTUDIO DE MEDIDAS CORPORALES, PESO VIVO Y CONDICIÓN CORPORAL DE BOVINOS HEMBRAS DE LA RAZA NELORE (Bos taurus indicus POR 12 MESES ESTUDO DE MEDIDAS CORPORAIS, PESO VIVO E CONDIÇÃO CORPORAL DE FÊMEAS DA RAÇA NELORE (Bos taurus indicus AO LONGO DE 12 MESES

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    Arcádio de Los Reys Borjas

    2008-04-01

    Full Text Available Four hundred and eighty cattle were used to verify alterations and correlations among corporal measures in Nelore zebu herd with cows and heifers. Body weight and length, corporal condition, heart girth, withers height and hip measures were evaluated. Eight collections were accomplished along the months of October of 2002 and October of 2003. In heifers there was increase of the averages of corporal measures with significant difference (p <0,05 among collections only the heart girth was different (p <0,05 in cows. The relationship between the body weight and body condition with the time were quadratic parallel curves (p <0,001. There were correlations among lineal measures body with hip measures (p<0,001 except for heart girth with hip length. The correlations of body weight and body condition among body measures were significant (p<0,001 except body condition with hip length in cows. It could be concluded that there was a growing variation of the body measures in heifers in the experimental period. The body weight, the body condition and heart girth were related with different periods of the year that the evaluation was accomplished. In cows the variations along the year were of 14,79%, 31,53% and 6,74%, respectively. The isquiun – iliun external measures, as height and width were correlated with size measures and weight. The body weight and body condition in heifers behave in way similar to cows. Further researches in relationship among body measures, body weight and body condition with productive and reproductive aspect are necessary. Con el objetivo de verificar alteraciones y correlaciones entre medidas corporales en un rebaño bovino de vaquillonas y vacas de la raza Nelore. Evaluaron-se 487 hembras en peso vivo, condición corporal, perímetro toráxico, largura corporal, altura de la cruz y medidas da anca. Fueron realizadas ocho coletas a lo largo de los meses de octubre de 2002 y octubre de 2003. En las vaquillonas hubo un aumento de

  19. Cocaine-induced vasculitis: is this a new trend?

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    García Pérez MR

    2013-10-01

    could not complete the treatment because she escaped from the hospital before finishing her treatment.Keywords: levamisole, cocaine-induced vasculitis, vasculitis, P-ANCA, C-ANCA

  20. GLOBAL FINANCIAL CRISIS AND UNIT-LINKED INSURANCE MARKETS EFFICIENCY: EMPIRICAL EVIDENCE FROM CENTRAL AND EASTERN EUROPEAN COUNTRIES

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    Botoş Horia Mircea

    2012-12-01

    Full Text Available This paper empirically investigates the impact of the Global financial crisis on the efficiency of four Central and Eastern European emerging unit-linked insurance markets, applying the automatic variance ratio (AVR test of Kim (2009 and variance ratio tests using ranks and signs by Wright (2000 for entire, pre-crisis and crisis periods. This study contributes to the existing literature on efficient market hypothesis with several distinct features: it provides a systematic review of the weak-form market efficiency literature that examines return predictability of the daily ING unit-linked funds prices; also the article aims at monitoring any improvement in the degree of efficiency in time and also examines the relative efficiency of unit-linked insurance markets in pre-crisis and crisis periods. Unit linked insurance are life insurance policies with investment component. In the literature there are few studies investigating the effects of a financial crisis on the potential of predictability and implicitly on the degree of efficiency of financial markets. The occurrence of a market crash or financial crisis is a possible contributing factor of market inefficiency. Most of the studies are focused on the Asian crisis in 1997: Holden et al. (2005 examined the weak-form efficiency of eight emerging Asian stock markets using VR tests before, during and after the Asian crisis; Kim and Shamsuddin (2008 used three different types of multiple VR tests for nine Asian stock markets; the findings reported by Lim et al. (2008 are consistent with those reported by Cheong et al. (2007, in which the highest inefficiency occurs during the crisis period. Todea and Lazar (2010 investigated the effects of the Global crisis on the relative efficiency of ten CEE stock markets, using Generalized Spectral test of Escanciano and Velasco (2006. Wright (2000 proposes the alternative non-parametric variance ratio tests using ranks and signs of return and demonstrates that

  1. Cómo y para qué se ha instaurado el discurso de la maximización de beneficios en la esfera psicoterapéutica: análisis de las evaluaciones de costo-beneficio

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    Paula Ascorra

    2008-11-01

    Full Text Available

    Lo que me motiva a escribir el presente artículo dice relación con la posibilidad de poder analizar el por qué y para qué se han introducido herramientas administrativas a la evaluación de los procesos psicoterapéuticos. No me motiva investigar qué tipo de intervención psicoterapéutica puede resultar más adecuada para tal tipo de problema psicológico; sino construir algunas hipótesis que justifiquen la introducción de constructos tales como eficiencia, maximización de beneficios, eficacia, efectividad, etc. al campo psicoterapéutico.También me interesa cuestionar la utilidad de instaurar ciertos modelos de explicación derivados de las “ciencias duras” al campo de la salud mental.

    Para cumplir el propósito que me propongo, analizaré qué es lo que se entiende por investigaciones en análisis de costo y sugeriré quiénes legitiman la introducción de este tipo de dispositivo social en nuestras formas de vida y para qué lo hacen. Posteriormente abordaré las investigaciones publicadas tomando como referencia el metaanálisis realizado por Gabbard, Lazar, Hornberger y Spiegel en 1997, develando las falencias que presentan los estudios publicados. Por último, en la discusión final voy a sostener que este tipo de estudio constituye un dispositivo social -validado por la administración y la economía, principalmente- mediante el cual se favorecen los intereses de actores sociales específicos, entre los cuales destacan las compañías aseguradoras de salud, el Estado y aquellas intervenciones psicoterapéuticas que poseen un marcado enfoque médico; en particular, la psiquiatría.

  2. An ensemble model of QSAR tools for regulatory risk assessment.

    Science.gov (United States)

    Pradeep, Prachi; Povinelli, Richard J; White, Shannon; Merrill, Stephen J

    2016-01-01

    Quantitative structure activity relationships (QSARs) are theoretical models that relate a quantitative measure of chemical structure to a physical property or a biological effect. QSAR predictions can be used for chemical risk assessment for protection of human and environmental health, which makes them interesting to regulators, especially in the absence of experimental data. For compatibility with regulatory use, QSAR models should be transparent, reproducible and optimized to minimize the number of false negatives. In silico QSAR tools are gaining wide acceptance as a faster alternative to otherwise time-consuming clinical and animal testing methods. However, different QSAR tools often make conflicting predictions for a given chemical and may also vary in their predictive performance across different chemical datasets. In a regulatory context, conflicting predictions raise interpretation, validation and adequacy concerns. To address these concerns, ensemble learning techniques in the machine learning paradigm can be used to integrate predictions from multiple tools. By leveraging various underlying QSAR algorithms and training datasets, the resulting consensus prediction should yield better overall predictive ability. We present a novel ensemble QSAR model using Bayesian classification. The model allows for varying a cut-off parameter that allows for a selection in the desirable trade-off between model sensitivity and specificity. The predictive performance of the ensemble model is compared with four in silico tools (Toxtree, Lazar, OECD Toolbox, and Danish QSAR) to predict carcinogenicity for a dataset of air toxins (332 chemicals) and a subset of the gold carcinogenic potency database (480 chemicals). Leave-one-out cross validation results show that the ensemble model achieves the best trade-off between sensitivity and specificity (accuracy: 83.8 % and 80.4 %, and balanced accuracy: 80.6 % and 80.8 %) and highest inter-rater agreement [kappa ( κ ): 0

  3. Microorganismos probióticos y salud

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    María Teresa Sánchez

    2015-01-01

    Full Text Available Objetivo: Mostrar los beneficios de los microorganismos probióticos sobre la salud y su aceptación por parte del consumidor, así como hacer una recopilación de todos los productos probióticos disponibles en el mercado farmacéutico. Material y métodos: Se realizó un estudio del mercado farmacéutico en relación a las formas farmacéuticas con microorganismos probióticos existentes y su evolución en los últimos años. La clasificación de todos los productos probióticos se llevó a cabo en función de la forma farmacéutica en la que se presentan;cada producto irá acompañado de la dosis de microorganismos probióticos que contiene, expresada como Unidades Formadoras de Colonias (UFC. Resultados: Es cada vez mayor el número de cepas probióticas aisladas y los beneficios mostrados sobre la salud del hombre. Encontramos gran diversidad de productos probióticos disponibles en oficinas de farmacia como consecuencia de una demanda cada vez mayor por parte del consumidor; no obstante, cabe resaltar el hecho de que muchos de ellos carecen en envase de información necesaria, por ejemplo, la dosis contenida. Conclusiones: El interés por parte de la industria farmacéutica en lazar nuevas formas farmacéuticas contenidas en microorganismos probióticos será cada vez mayor e irá ligado a la necesidad de una reglamentación específica para estos productos. Muchos de ellos no contienen la dosis mínima requerida para obtener un efecto beneficioso en la salud lo que supone una publicidad engañosa para el consumidor, por tanto, deberían ser retirados del mercado, publicitando únicamente aquellos que contengan una dosis terapéutica y cuyos efectos estén avalados por diferentes ensayos clínicos.

  4. Purpura, petechiae, and bullae as first signs of juvenile granulomatosis with polyangiitis.

    Science.gov (United States)

    Rawn, Saara; Miettunen, Paivi; Brown, Holly A; Schmeling, Heinrike

    2014-12-01

    We present a case of a 14-year-old girl who had a severe form of granulomatosis with polyangiitis (GPA) with extensive dermatological involvement, whose initial presentation was nonspecific leading to diagnostic confusion and initial consideration of infectious and other vasculitis causes. The patient presented with fever, congestion, malaise, and sinus pain. She was diagnosed with bacterial sinusitis and treated with antibiotics. Within weeks, she developed abdominal pain, hematuria, migratory arthritis, and palpable purpura and was diagnosed with Henoch-Schonlein purpura. She went on to develop hemoptysis and progression of the rash into erosive bullae. Investigations revealed that she was ANCA positive and had pauci-immune glomerulonephritis. Given her upper airway, pulmonary and renal involvement, and antineutrophil cytoplasmic antibodies positivity, a definitive diagnosis of a severe form of GPA was made. GPA is a chronic relapsing, life threatening vasculitis that predominantly affects small vessels. Our case demonstrates that GPA can present initially with nonspecific symptoms, including extensive dermatological involvement, leading to diagnostic confusion, and delays in treatment. In the case of a severe peripheral rash in the juvenile population and/or resistant upper airway symptoms, it is vital to consider a diagnosis of GPA to avoid serious organ or life threatening consequences.

  5. Ischemia-induced glomerular parietal epithelial cells hyperplasia: Commonly misdiagnosed cellular crescent in renal biopsy.

    Science.gov (United States)

    Zeng, Yeting; Wang, Xinrui; Xie, Feilai; Zheng, Zhiyong

    2017-08-01

    Ischemic pseudo-cellular crescent (IPCC) that is induced by ischemia and composed of hyperplastic glomerular parietal epithelial cells resembles cellular crescent. In this study, we aimed to assess the clinical and pathological features of IPCC in renal biopsy to avoid over-diagnosis and to determine the diagnostic basis. 4 IPCC cases diagnosed over a 4-year period (2012-2015) were evaluated for the study. Meanwhile, 5 cases of ANCA-associated glomerulonephritis and 5 cases of lupus nephritis (LN) were selected as control. Appropriate clinical data, morphology, and immunohistochemical features of all cases were retrieved. Results showed that the basement membrane of glomerulus with IPCC appeared as a concentric twisted ball, and glomerular cells of the lesion were reduced even entirely absent, and the adjacent afferent arterioles showed sclerosis or luminal stenosis. Furthermore, immune globulin deposition, vasculitis, and fibrinous exudate have not been observed in IPCC. While the cellular crescents showed diverse characteristics in both morphology and immunostaining in the control group. Therefore, these results indicated that IPCC is a sort of ischemic reactive hyperplasia and associated with sclerosis, stenosis, or obstruction of adjacent afferent arterioles, which is clearly different from cellular crescents result from glomerulonephritis. Copyright © 2017 Elsevier GmbH. All rights reserved.

  6. Cocaine-Levamisole-Induced Vasculitis/Vasculopathy Syndrome.

    Science.gov (United States)

    Marquez, Javier; Aguirre, Lina; Muñoz, Carolina; Echeverri, Andres; Restrepo, Mauricio; Pinto, Luis F

    2017-06-01

    To understand the clinical spectrum of cocaine-levamisole-induced vasculitis. Worldwide recreational drug consumption is high among the adult population from various social strata. The use of cocaine with levamisole, a frequently added antiparasitic diluent, favors the manifestations of vasculitic lesions, especially in the skin. New insights into immunological mechanisms involved in the pathogenesis of the disease. There are still many unknown aspects in the pathogenesis of this disease, such as the immune system interaction with p-ANCAs and the release of inflammatory NETs (neutrophil extracellular traps), which are the origin of auto-antigens and tissue damage, manifesting as vasculitic purpura on the skin. The clinical presentation constitutes a challenge for the clinician to be able to distinguish it from small-vessel vasculitides. This paper intends to improve the understanding of this condition, exhibiting the broad clinical spectrum of local and systemic manifestations of cocaine-levamisole-induced vasculitis, to facilitate a timely diagnosis, in order to take corrective measures and avoid sequelae, along with tissue damage and the consequent deformities and permanent scars.

  7. Infections and vasculitis.

    Science.gov (United States)

    Thomas, Konstantinos; Vassilopoulos, Dimitrios

    2017-01-01

    To review recent evidence for infection rates in patients with systemic vasculitides, the role of specific infectious agents in the pathogenesis of vasculitis and recent breakthroughs in the treatment of virus-associated vasculitides. In well designed recent studies, infections were found to be common during the first 6-12 months in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) and giant cell arteritis (GCA) and to contribute significantly to increased mortality during this period. New therapeutic schemes with lower cyclophosphamide doses and shorter corticosteroid courses were associated with decreased infectious rates in elderly patients with AAV whereas a prednisone dose greater than 10 mg/day at the end of the first year were associated with increased infectious-related mortality in patients with GCA. Recently, a potential role for varicella zoster virus in GCA pathogenesis has been proposed but more data are needed in order to establish a causal relationship. Finally, preliminary data show excellent short-term efficacy and safety of the new, interferon-free, oral antiviral agents in the treatment of hepatitis C virus-associated cryoglobulinemic vasculitis. Infections continue to be one of the main causes of mortality in patients with systemic vasculitides, emphasizing the need for safer immunosuppressive therapies and appropriate prophylaxis.

  8. Advances in the use of biologic agents for the treatment of systemic vasculitis

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    Chung, Sharon A.; Seo, Philip

    2010-01-01

    Purpose of review Due to the well-known toxicities of cyclophosphamide, substantial interest exists in finding other therapies to treat primary systemic vasculitis. Biologic agents have been proposed as an alternative to cyclophosphamide for these disorders because of their recent success in treating other rheumatic diseases. This article reviews the current state-of-the-art with regards to the use of biologic agents as a treatment for systemic vasculitis. Recent findings The greatest amount of experience with these agents for the treatment of systemic vasculitis is with anti-tumor necrosis factor agents, pooled intravenous immunoglobulin, and anti-B cell therapies such as rituximab. Intravenous immunoglobulin is already a standard therapy for Kawasaki's disease, but should also be considered for the treatment of ANCA-associated vasculitis when standard therapies are either ineffective or contraindicated. Early experience with tumor necrosis factor inhibitors indicates that they may be effective for the treatment of Takayasu's arteritis, but their role in the treatment of other forms of vasculitis remains controversial. Early experience with rituximab for the treatment of several forms of vasculitis has been quite promising, but must be confirmed by ongoing randomized clinical trials. Summary Biologic agents represent the next evolution in treatment for the primary systemic vasculitides. Greater understanding of these diseases has allowed use to move further away from non-specific, highly toxic therapies towards a more directed approach. As our experience with these agents increases, they will likely form the keystone of treatment in the near future. PMID:19077713

  9. Neutrophil extracellular traps in vasculitis, friend or foe?

    Science.gov (United States)

    Söderberg, Daniel; Segelmark, Mårten

    2018-01-01

    Neutrophil extracellular traps (NETs) can be found at the sites of vascular lesions and in the circulation of patients with active small vessel vasculitis. Neutrophils from vasculitis patients release more NETs in vitro, and NETs have properties that can harm the vasculature both directly and indirectly. There are several ways to interfere with NET formation, which open for new therapeutic options. However, there are several types of NETs and different mechanisms of NET formation, and these might have different effects on inflammation. Here we review recent findings regarding the pathogenesis and therapeutic potentials of NETs in vasculitis. Experimental mouse models support a role for NETs in promoting vascular damage, where histones and mitochondrial DNA appear to be driving forces. Impaired formation of NETs, however, in an SLE-like mouse model leads to more severe disease, suggesting that NETs can be important in limiting inflammation. Studies on drug-induced vasculitis reveal that levamisole can induce NETosis via muscarinic receptors, predisposing for the generation of autoantibodies, including antineutrophil cytoplasmic autoantibodies (ANCA). This supports the notion that NETs can bridge the innate and adaptive immune systems. NETs can participate in the pathogenesis of vasculitis, but in some models there also seem to be protective effects of NETs. This complexity needs further evaluation with experimental models that are as specific as possible for human primary vasculitis.

  10. Patterns of glomerulonephritis with crescents: Experience at a tertiary medical center in Saudi Arabia

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    Turki Al-Hussain

    2017-01-01

    Full Text Available A series of 78 cases of glomerulonephritis (GN, in which renal biopsy revealed changes of GN associated with crescent formation, were reviewed. Renal pathology findings were correlated with clinical features including patient’s age, renal function, and serologic findings. In most of the cases (71.8%, the crescents were due to immune complex-mediated GN. This was followed by pauci-immune GN (20.5% and anti-glomerular basement membrane antibody (GBM GN (7.7%. The percentage of glomeruli with crescents was the highest in cases of anti-GBM disease (mean of 93.3%, followed by pauci-immune GBM (mean of 48.2% and immune complex GN (30.9%. In cases with the pauci- immune GN, there were additional features of glomerular injury including fibrinoid necrosis, disruption of the GBM, and rupture of Bowman’s capsule. These changes were generally more pronounced in a subset of pauci-immune GN associated with serum elevation of antineutrophil cytoplasmic antibody (c-ANCA. In biopsies from patient with immune complex disease, systemic lupus erythematosus was the most common cause of crescentic GN.

  11. Anti-glomerular basement membrane glomerulonephritis in an HIV positive patient: case report

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    Eduardo José Bellotto Monteiro

    Full Text Available We report on a case of a patient with HIV infection, diagnosed 18 months prior to the development of an anti-glomerular basement membrane (anti-GBM rapidly progressive glomerulonephritis; this is probably the first report of such an association. A 30-year-old white man presented with elevation of serum creatinine (1.3 - 13.5 mg/dL within one month. At admission, the urinalysis showed proteinuria of 7.2 g/L and 8,000,000 erythrocytes/mL. Renal biopsy corresponded to a crescentic diffuse proliferative glomerulonephritis mediated by anti-GBM, and serum testing for anti-GBM antibodies was positive; antinuclear antibodies (ANA and anti-neutrophilic cytoplasmic antibodies (ANCA were also positive. The patient underwent hemodyalisis and was treated with plasmapheresis, cyclophosphamide and prednisone. The association described here is not casual, as crescentic glomerulonephritis is not common in HIV-positive patients, anti-GBM glomerulonephritis is rare and anti-GBM antibodies are frequently observed in HIV-positive subjects when compared to the overall population. Based on the current case and on the elevated frequency of the positivity for such antibodies in this group of patients, it is advisable to be aware of the eventual association between these two conditions and to promote an active search for anti-GBM antibodies and early diagnosis of eventual urinary abnormalities in HIV-positive subjects, considering the severity of anti-GBM glomerulonephritis.

  12. Predicting Outcome in Patients with Anti-GBM Glomerulonephritis.

    Science.gov (United States)

    van Daalen, Emma E; Jennette, J Charles; McAdoo, Stephen P; Pusey, Charles D; Alba, Marco A; Poulton, Caroline J; Wolterbeek, Ron; Nguyen, Tri Q; Goldschmeding, Roel; Alchi, Bassam; Griffiths, Meryl; de Zoysa, Janak R; Vincent, Beula; Bruijn, Jan A; Bajema, Ingeborg M

    2018-01-06

    Large studies on long-term kidney outcome in patients with anti-glomerular basement membrane (anti-GBM) GN are lacking. This study aimed to identify clinical and histopathologic parameters that predict kidney outcome in these patients. This retrospective analysis included a total of 123 patients with anti-GBM GN between 1986 and 2015 from six centers worldwide. Their kidney biopsy samples were classified according to the histopathologic classification for ANCA-associated GN. Clinical data such as details of treatment were retrieved from clinical records. The primary outcome parameter was the occurrence of ESRD. Kidney survival was analyzed using the log-rank test and Cox regression analyses. The 5-year kidney survival rate was 34%, with an improved rate observed among patients diagnosed after 2007 ( P =0.01). In patients with anti-GBM GN, histopathologic class and kidney survival were associated ( P GBM GN. Kidney outcome has improved during recent years; the success rate doubled after 2007. This article contains a podcast at https://www.asn-online.org/media/podcast/CJASN/2017_11_21_CJASNPodcast_18_1_v.mp3. Copyright © 2018 by the American Society of Nephrology.

  13. Determination of the δ13C (per mille) isotopic rate of the Brazilian beer pilsen type

    International Nuclear Information System (INIS)

    Rossete, Alexssandra L.R.M.; Bendassolli, Jose Albertino; Lopes, Fabio; Martinelli, Luiz Antonio

    2002-01-01

    Beer has been consumed in Brazil since the XIX century. However, at that time, this beverage was imported from England, and the first Brazilian breweries just appeared at the end of the XX century. Basically, beer is made from: malt of barley, Humulus lupulus, water and yeast. In this work, twenty brands of national beer (Pilsen type), and also two different cereals, barley, a C3 metabolism plant, corn, a C4 plant, were analysed to determine their isotope composition δ 13 C (per mille) utilizing a mass spectrometer Delta Plus Finigam and Anca S.L. An aliquot of each sample was transferred using a capillary of glass to a tin capsule contends a small amount of sorbitol. The isotope compositions, δ 13 C, of barley and corn samples determined were -27.2 and -11.5 per mille, respectively. These results were used to calculate the equation of mixture model. Considering the beer samples, the δ 13 C ranged from -18.3 to -23.1 per mille; this result means that the percentage of C3 plant is from 43.3 to 73.9 per mille. In this manner, it is possible to concluded that all beer samples has malted cereals (C4 plant), as far as the low values of C3 plants were presented in these samples as shown through the equation of mixtures model. (author)

  14. Case report of a 28-year-old male with the rapid progression of steroid-resistant central nervous system vasculitis diagnosed by a brain biopsy.

    Science.gov (United States)

    Takahashi, Keigo; Sato, Hideki; Hattori, Hidenori; Takao, Masaki; Takahashi, Shinichi; Suzuki, Norihiro

    2017-09-30

    A 28-year-old Japanese male without a significant past medical history presented with new-onset generalized clonic seizure and headache. A brain MRI revealed multiple enhanced lesions on both cerebral hemispheres. Laboratory exams showed no evidence of systemic inflammation or auto-immune antibodies such as ANCAs. Despite four courses of high-dose methylprednisolone pulse therapy and five treatments with plasmapheresis, his symptoms worsened and the MRI lesions progressed rapidly. During these treatments, we performed a targeted brain biopsy, that revealed histological findings consistent with a predominant angiitis of parenchymal and subdural small vessels. He was provided with diagnosis of central nervous system vasculitis (CNSV). Subsequent cyclophosphamide pulse therapy enabled a progressive successful improvement of his symptoms. While diagnostic methods for CNSV remain controversial, histological findings are thought to be more useful in obtaining a more definitive diagnosis than findings in image studies, such as MRI and angiography. We suggest that a brain biopsy should be considered during the early period of cases with suspected CNSV and rapid clinical deterioration. We also detected human herpesvirus 7 (HHV-7) using PCR technology in brain biopsy specimens, however the relationship between CNSV and HHV-7 infection is unknow.

  15. Wegeners Granulomatosis

    International Nuclear Information System (INIS)

    Canas Davila, Carlos Alberto; Restrepo Suarez, Jose Felix; Iglesias Gamarra, Antonio

    2001-01-01

    To review the recent medical literature with regard to the etiopathogenic and clinical aspects of the Wegeners Granulomatosis (WG), We carried out a search in the Medline database (1990-2000) that comprises topics related with etiology, epidemiology, pathology, and clinical aspect of WG. 650 abstracts were studied, finding that 125 of them informed topics related with the topic that we wanted to study. Then we got the articles. Some bibliographical references of these articles were considered fundamental for our objectives and we decided to get it too. The articles were classified according to their objectives and execution strategies, as they were revisions, original articles or cases report. We proceeded to their reading and analysis, for the later elaboration of the revision. We made a selection of information based in the following model: definition, history, epidemiology, etiopathogenesis, pathology, clinical aspects, course and evolution, diagnosis criteria, activity markers and treatment. WG is an infrequent disease The pathogenic and the therapeutic aspects are motives of recent and important advances in the medicine research, specially the state of the knowledge of antineutrophil cytoplasmic antibodies (ANCAs) and therapeutic tools which had decrease the mortality of this disease

  16. in Children

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    Monika Jabłońska-Jesionowska

    2016-12-01

    Full Text Available Introduction. Chronic rhinitis in children may have different causes, both local – with changes being present only in the nasalcavity – or systemic, with nasal congestion as one of the symptoms of a bigger clinical picture.Aim. the aim of this study was to draw attention to a very rare congenital cause of chronic rhinitis in children – which is hypohidrotic ectodermal dysplasia.Material and methods. A 6-month-old boy was admitted to the department of pediatric otolaryngology of Warsaw medicalUniversity due to chronic nasal obstruction present from birth. Clinical investigation included anterior and posterior rhinoscopy and fiberoscopy of nasopharynx. the mri was also performed before admission. Complete blood count, serum iron level,serum thyroid hormones and level of igG, igA, igm were examined to exclude anaemia, ozaena and hypothyroidism. Antinuclear antibodies (AnA and antineutrophil cytoplasmic antibodies (AnCA tests were also ordered to exclude granulomatosiswith polyangiitis. next, a mucosal biopsy of the nasal cavity was performed to exclude primary ciliary dyskinesia. Allergic pricktests were also performed.Results. After genetic tests, hypohidrotic ectodermal dysplasia was diagnosed.Conclusions. 1. every case of chronic nasal congestion in children requires not only adequate treatment, but also thoroughclinical investigation. 2. nasal obstruction may be due to local causes, systemic diseases and genetic disorders. 3. hypohidroticectodermal dysplasia is a very rare genetic disorder that causes severe, even life threatening symptoms, one of which is chronicrhinitis.

  17. Predictors of Outcome in Ulcerative Colitis.

    Science.gov (United States)

    Waterman, Matti; Knight, Jo; Dinani, Amreen; Xu, Wei; Stempak, Joanne M; Croitoru, Kenneth; Nguyen, Geoffrey C; Cohen, Zane; McLeod, Robin S; Greenberg, Gordon R; Steinhart, A Hillary; Silverberg, Mark S

    2015-09-01

    Approximately 80% of patients with ulcerative colitis (UC) have intermittently active disease and up to 20% will require a colectomy, but little data available on predictors of poor disease course. The aim of this study was to identify clinical and genetic markers that can predict prognosis. Medical records of patients with UC with ≥5 years of follow-up and available DNA and serum were retrospectively assessed. Immunochip was used to genotype loci associated with immune mediated inflammatory disorders (IMIDs), inflammatory bowel diseases, and other single nucleotide polypmorphisms previously associated with disease severity. Serum levels of pANCA, ASCA, CBir1, and OmpC were also evaluated. Requirement for colectomy, medication, and hospitalization were used to group patients into 3 prognostic groups. Six hundred one patients with UC were classified as mild (n = 78), moderate (n = 273), or severe disease (n = 250). Proximal disease location frequencies at diagnosis were 13%, 21%, and 30% for mild, moderate, and severe UC, respectively (P = 0.001). Disease severity was associated with greater proximal extension rates on follow-up (P 40 and proximal disease location were associated with severe UC (odds ratios = 1.94 and 2.12, respectively). None of the single nucleotide polypmorphisms or serum markers tested was associated with severe UC, proximal disease extension or colectomy. Older age and proximal disease location at diagnosis, but not genetic and serum markers, were associated with a more severe course. Further work is required to identify biomarkers that will predict outcomes in UC.

  18. [Role of anti c-mpl antibody in systemic lupus erythematosus with thrombocytopenia].

    Science.gov (United States)

    Yang, Tuo; Huang, Ci Bo; Lai, Bei; Zhao, Li Ke; Chen, Ying Juan; Zhao, Yue Tao; Zhang, Chun Mei; Zeng, Xiao Feng

    2012-04-18

    To determine whether anti-thrompoietin receptor (TPO-R, c-mpl) antibody contributes to thrombocytopenia in systemic lupus erytematosus (SLE) and explore the pathogenic role of this antibody. Sera from 24 SLE patients with thrombocytopenia, 27 SLE patients having normal platelet counts with a history of thrombocytopenia, 18 SLE patients with neither thrombocytopenia nor post thrombocytopenia and 18 healthy controls were collected. Anti c-mpl antibodies were detected by an indirected ELISA assay. The serum TPO levels were measured by an ELISA assay. Clinical findings, autoantibody profiles, and SLEDAI were evaluated. Serum anti c-mpl antibodies were detected in 18.8% of the SLE patientis. The frequency of this antibody in SLE with thrombocytopenia, SLE with a history of thrombocytopenia and SLE without thrombocytopenia were of no difference (P=0.600). In the patients with anti c-mpl antibodies, their platelet counts were decreased(P=0.025) and serum TPO levels elevated(P=0.038) than those in the patients without, while there were no differences between the two groups in C3, C4, ESR, CRP level, the frequency of ANA, dsDNA, ANCA and SLEDAI. Anti c-mpl antibody contributes to SLE-associated thrombocytopenia by functionally blocking an interaction between thrombopoietin and c-mpl, which might inhibit TPO-dependent megakaryocyte proliferation and differentiation.

  19. Cetuximab-Associated Crescentic Diffuse Proliferative Glomerulonephritis

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    Sukesh Manthri

    2017-01-01

    Full Text Available Cetuximab-induced nephrotoxicity is very rare, occurring in less than 1% of colorectal cancer patients and not defined in other populations. We report a rare case of crescentic diffuse proliferative glomerulonephritis (GN that developed in close temporal association with cetuximab treatment. A 65-year-old female recently completed chemotherapy with cetuximab treatment for moderately differentiated oral squamous cell carcinoma. She was admitted with acute renal failure and nephrotic-range proteinuria. Laboratory data showed serum creatinine of 6.6 mg/dl and urinalysis showed proteinuria, moderate hemoglobinuria, hyaline casts (41/LPF, WBC (28/HPF, and RBC (81/HPF. Serologic studies were negative for ANA, anti-GBM, ANCA, hepatitis B, and hepatitis C. Serum C3 and C4 level were normal. Renal biopsy showed crescentic diffuse proliferative GN with focal features of thrombotic microangiopathy. Patient was started on cyclophosphamide and steroids. Her renal function did not improve on day 8 and she was started on hemodialysis. Previous reports suggest that EGFR-targeting medications can possibly trigger or exacerbate an IgA-mediated glomerular process leading to renal failure. This case suggests that cetuximab therapy may have triggered or exacerbated a severe glomerular injury with an unfavorable outcome. Treating physicians should maintain a high degree of caution and monitor renal function in patients on EGFR inhibitors.

  20. Healing rate and autoimmune safety of full-thickness wounds treated with fish skin acellular dermal matrix versus porcine small-intestine submucosa: a noninferiority study.

    Science.gov (United States)

    Baldursson, Baldur Tumi; Kjartansson, Hilmar; Konrádsdóttir, Fífa; Gudnason, Palmar; Sigurjonsson, Gudmundur F; Lund, Sigrún Helga

    2015-03-01

    A novel product, the fish skin acellular dermal matrix (ADM) has recently been introduced into the family of biological materials for the treatment of wounds. Hitherto, these products have been produced from the organs of livestock. A noninferiority test was used to compare the effect of fish skin ADM against porcine small-intestine submucosa extracellular matrix in the healing of 162 full-thickness 4-mm wounds on the forearm of 81 volunteers. The fish skin product was noninferior at the primary end point, healing at 28 days. Furthermore, the wounds treated with fish skin acellular matrix healed significantly faster. These results might give the fish skin ADM an advantage because of its environmental neutrality when compared with livestock-derived products. The study results on these acute full-thickness wounds might apply for diabetic foot ulcers and other chronic full-thickness wounds, and the shorter healing time for the fish skin-treated group could influence treatment decisions. To test the autoimmune reactivity of the fish skin, the participants were tested with the following ELISA (enzyme-linked immunosorbent assay) tests: RF, ANA, ENA, anti ds-DNA, ANCA, anti-CCP, and anticollagen I and II. These showed no reactivity. The results demonstrate the claims of safety and efficacy of fish skin ADM for wound care. © The Author(s) 2015.

  1. Performance analysis of automated evaluation of Crithidia luciliae-based indirect immunofluorescence tests in a routine setting - strengths and weaknesses.

    Science.gov (United States)

    Hormann, Wymke; Hahn, Melanie; Gerlach, Stefan; Hochstrate, Nicola; Affeldt, Kai; Giesen, Joyce; Fechner, Kai; Damoiseaux, Jan G M C

    2017-11-27

    Antibodies directed against dsDNA are a highly specific diagnostic marker for the presence of systemic lupus erythematosus and of particular importance in its diagnosis. To assess anti-dsDNA antibodies, the Crithidia luciliae-based indirect immunofluorescence test (CLIFT) is one of the assays considered to be the best choice. To overcome the drawback of subjective result interpretation that inheres indirect immunofluorescence assays in general, automated systems have been introduced into the market during the last years. Among these systems is the EUROPattern Suite, an advanced automated fluorescence microscope equipped with different software packages, capable of automated pattern interpretation and result suggestion for ANA, ANCA and CLIFT analysis. We analyzed the performance of the EUROPattern Suite with its automated fluorescence interpretation for CLIFT in a routine setting, reflecting the everyday life of a diagnostic laboratory. Three hundred and twelve consecutive samples were collected, sent to the Central Diagnostic Laboratory of the Maastricht University Medical Centre with a request for anti-dsDNA analysis over a period of 7 months. Agreement between EUROPattern assay analysis and the visual read was 93.3%. Sensitivity and specificity were 94.1% and 93.2%, respectively. The EUROPattern Suite performed reliably and greatly supported result interpretation. Automated image acquisition is readily performed and automated image classification gives a reliable recommendation for assay evaluation to the operator. The EUROPattern Suite optimizes workflow and contributes to standardization between different operators or laboratories.

  2. History of primary vasculitis in Latin America.

    Science.gov (United States)

    Iglesias Gammara, Antonio; Coral, Paola; Quintana, Gerardo; Toro, Carlos E; Flores, Luis Felipe; Matteson, Eric L; Restrepo, José Félix

    2010-03-01

    A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge.

  3. Elicitation of expert prior opinion: application to the MYPAN trial in childhood polyarteritis nodosa.

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    Lisa V Hampson

    Full Text Available Definitive sample sizes for clinical trials in rare diseases are usually infeasible. Bayesian methodology can be used to maximise what is learnt from clinical trials in these circumstances. We elicited expert prior opinion for a future Bayesian randomised controlled trial for a rare inflammatory paediatric disease, polyarteritis nodosa (MYPAN, Mycophenolate mofetil for polyarteritis nodosa.A Bayesian prior elicitation meeting was convened. Opinion was sought on the probability that a patient in the MYPAN trial treated with cyclophosphamide would achieve disease remission within 6-months, and on the relative efficacies of mycophenolate mofetil and cyclophosphamide. Expert opinion was combined with previously unseen data from a recently completed randomised controlled trial in ANCA associated vasculitis.A pan-European group of fifteen experts participated in the elicitation meeting. Consensus expert prior opinion was that the most likely rates of disease remission within 6 months on cyclophosphamide or mycophenolate mofetil were 74% and 71%, respectively. This prior opinion will now be taken forward and will be modified to formulate a Bayesian posterior opinion once the MYPAN trial data from 40 patients randomised 1:1 to either CYC or MMF become available.We suggest that the methodological template we propose could be applied to trial design for other rare diseases.

  4. “The NET outcome”: are neutrophil extracellular traps of any relevance to the pathophysiology of autoimmune disorders in childhood?

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    Stavros Giaglis

    2016-09-01

    Full Text Available Neutrophil extracellular trap (NET formation represents a form of cell death distinct from apoptosis or necrosis, by which invading pathogens are simultaneously entangled and potentially eliminated. Increased NET formation is observed in systemic lupus erythematosus (SLE, rheumatoid arthritis (RA, antineutrophil cytoplasmic antibody (ANCA-associated and small vessel vasculitis (SVV, antiphospholipid antibody syndrome (APS and psoriasis. NETs contribute to the pathogenesis of autoimmunity by exposing cryptic autoepitopes, which may facilitate the generation of autoantibodies, induce the production of interferons and activate the complement cascade. In SLE, augmented disease activity and renal disease are associated with increased NET formation, so that NETs could serve as a marker for the monitoring of disease activity. NETs can additionally cause endothelial cell damage and death and stimulate inflammation in atheromatous plaques, adding to the accelerated atherosclerosis witnessed in autoimmune disease. Since NETs induce production of interferons, assessing the extent of NET formation might facilitate the prediction of IFN-alpha levels and identification of SLE patients with presumably better responses to anti-IFN-alpha therapies or other novel therapeutic concepts, such as N-acetyl-cysteine and inhibitors of DNase 1, and peptidylarginine deiminase 4 (PAD4, which also target NETs. In summary, the study of NETs provides a novel approach to the understanding of autoimmune disease pathogenesis and opens new vistas in the development of sensitive disease markers and therapies.

  5. Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease.

    Science.gov (United States)

    Hanioka, Yusuke; Yamagami, Keiko; Yoshioka, Katsunobu; Nakamura, Tomomi; Kishida, Masatsugu; Nakamura, Tomoyuki; Yamaguchi, Toshimasa; Koshimo, Naomi; Inoue, Takeshi; Imanishi, Masahito

    2012-01-01

    A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.

  6. Leukocytoclastic vasculitis: A window to systemic Churg Strauss syndrome

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    Sudhir V Medhekar

    2012-01-01

    Full Text Available A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE, Anti nuclear antibody (ANA positivity and negative antineutrophil cytoplasmic antibody (ANCA. Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS. This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.

  7. Optimal therapy and prospects for new medicines in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

    Science.gov (United States)

    Pagnoux, Christian; Groh, Matthieu

    2016-10-01

    The prevalence of eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome) is lower than that of other antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV's), and only a few randomized controlled trials have been conducted for this rare disease. However, recent international efforts have helped delineate the best treatment approach. At present, EGPA conventional therapy is by default similar to that of other AAVs. Limited, non-severe EGPA can initially be treated with glucocorticoids (GCs) alone. Patients with life-threatening manifestations and/or major organ involvement must receive a combination of GCs and an immunosuppressant, mainly cyclophosphamide. Remission can be achieved in >85% of patients with these first-line treatments, but vasculitis relapses occur in more than one-third of patients, and about 85% cannot stop GC treatment because of GC-dependent asthma and/or ENT manifestations. A few biologic agents, including rituximab or mepolizumab, are now under investigation after interesting preliminary results. Expert commentary: Treatment for EGPA still has several unmet needs. Several biologic agents are now under investigation in randomized controlled trials, but a few others should be considered soon. Their benefit should be demonstrated for devising more EGPA-tailored therapeutic strategies (ideally GC-free).

  8. A case report and literature review of Churg-Strauss syndrome presenting with myocarditis.

    Science.gov (United States)

    Qiao, Lu; Gao, Dengfeng

    2016-12-01

    Churg-Strauss syndrome (CSS) is a multisystem disorder characterized by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. Here we report a case of CSS presenting with acute myocarditis and heart failure and review the literature on CSS with cardiac involvement. A 59-year-old man with general fatigue, numbness of limbs, and a 2-year history of asthma was admitted to the department of orthopedics. Eosinophilia, history of asthma, lung infiltrates, peripheral neurological damage, and myocarditis suggested the diagnosis of CSS. Transthoracic echocardiography revealed a dilated hypokinetic left ventricle (left ventricular ejection fraction ∼40%) with mild segmental abnormalities in the septal and apical segments. By reviewing the present case reports, we concluded that (1) the younger age of CSS, the greater occurrence rate of complicating myocarditis and the poorer prognosis; (2) female CSS patients are older than male patients; (3) patients with cardiac involvement usually have a history of severe asthma; (4) markedly increased eosinophil count suggests a potential diagnosis of CSS (when the count increases to 20% of white blood cell counts or 8.1 × 109/L, eosinophils start to infiltrate into myocardium); and (5) negative ANCA status is associated with heart disease in CSS.

  9. Churg-Strauss vasculitis and idiopathic hypereosinophyl syndrome: role of molecular biology in the differential diagnosis of hypereosinophyl syndrome

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    A. d'Ascanio

    2011-09-01

    Full Text Available Objective: Hypereosinophilic syndromes are a heterogeneous group of uncommon disorders characterized by the presence of marked peripheral blood eosinophilia, tissue eosinophilia, or both, resulting in a wide variety of clinical manifestations, often without an identifiable cause. Churg-Strauss syndrome is a systemic vasculitis characterized by prominent peripheral eosinophilia, asthma and systemic involvement. The presence of mild to severe eosinophilia and systemic involvement raise the search of many trigger factor that need to be ruled out. Distinguishing CSS from idiopathic hypereosinophilic syndrome may be particularly challenging, especially in ANCA negative patients. Methods: The aim of the present study was to present a small case series of patients referred to a Rheumatology Unit for mild to severe eosinophilia and signs and symptoms of systemic involvement and to outline the clinical significance of molecular biology in the work-up of hypereosinophilia. Results: Eleven patients with moderate to severe peripheral eosinophylia, were referred to our Unit from 1996 to 2007. Female to male ratio was 7/4, mean age 40.54 (range 22-75. Three out of eleven patients resulted positive for molecular biology. The diagnosis of idiopathic hypereosinophylia was confirmed in one out of three on the basis of the clinical picture and bone marrow biopsy. Conclusions: Molecular biology may be useful in the screening and in the follow-up of a new hypereosinophylic patient.

  10. Churg-Strauss syndrome: A case report

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    Dinić Miroslav Ž.

    2013-01-01

    Full Text Available Introduction. Churg-Strauss syndrome (CSS is an allergic granulomatous angiitis, a rare disease of small and medium arteries and veins, associated with the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA. According to the American College of Rheumatology (ACR, there are four or more criteria out of six for the diagnosis: asthma, eosinophilia (> 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates, histological evidence of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. Case report. We reported a female patient, aged 80 years, with asthma for many decades and repeatedly verified eosinophilia in peripheral blood, in which CSS was suspected only after the occurrence of skin changes in the form of vesicles, vesiculopustule, purpuric macula, papule and petechiae. Further tests verified pulmonary infiltrates, paranasal sinusitis, extravascular eosinophils on histopathologic sample of skin tissue, and polyneuropathy. The treatment started with methylprednisolone (60 mg/d, with decreasing doses, and continued with pulse doses of cyclophosphamide (800 mg once monthly, also corticosteroid ointment for skin lesions. Conclusion. Despite long-standing pulmonary symptoms and laboratory findings of eosinophilia, the appearance of skin changes raised suspicion of possible CSS. Skin changes resolved and the patient was reffered to rheumatologist.

  11. Testing, testing, testing: an insidious hypereosinophilia

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    Livia Robbiolo

    2013-04-01

    Full Text Available Aim of the study: This case focuses on the difficulty in recognizing this rare entity characterized by systemic vasculitis in patients with history of asthma. Clinical case: We report a case of a 46-year-old man with history of allergic rhinitis and referred episodes of shortness of breath recognizing as acute bronchitis who presented with fever, skin eruption, peripheral eosonophilia, muscle weakness, abdominal pain and progressively dyspnea. Methods: Chest radiograph and computed tomography on admission showed consolidation in both lung fields and pericardial effusion. P-serum ANCA were positive. Neurological examination revealed mononeuritis multiplex. A skin biopsy was performed with presence of eosinophil granulocytes. The condition did not respond to antibiotics. He was supposed to have vasculitis and steroid treatment was started at a dose of 1 mg/kg; eosinophilia decreased but there was no clinical improvement. Abdominal pains were progressively more severe; emergency laparotomy revealed ileum infiammation, histopathological examination was consistent with Churg-Strauss syndrome.

  12. Anterior ischaemic optic neuropathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature.

    Science.gov (United States)

    Padovano, Ilaria; Pazzola, Giulia; Pipitone, Nicolò; Cimino, Luca; Salvarani, Carlo

    2014-01-01

    We report a 62-year-old man with mild fever, headache and acute visual loss in his right eye due to anterior ischaemic optic neuropathy (AION), followed a few days later by pain in the legs and left arm associated with numbness and weakness. Giant cell arteritis complicated by AION was suspected at the beginning and high-dose oral glucocorticoids were started. However, on the basis of the past medical history of nasal polyposis, asthma, and hypereosynophilia as well as of further investigations (biopsy of the nasal mucosa showing granulomatous inflammation with a rich eosinophilic infiltrate, electromyography demonstrating, mononeuritis multiplex and positive p-ANCA), eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, was diagnosed. Because visual acuity in the right eye deteriorated despite glucocorticoid therapy, pulse intravenous cyclophosphamide was started, subsequently replaced by oral azathioprine, while prednisone was slowly tapered. This treatment led to gradual improvement of the neurological symptoms, whereas the right visual impairment remained unchanged. EGPA-related AION is an uncommon lesion that is probably due to vasculitic involvement of posterior ciliary and/or chorioretinal arteries. The prognosis of established AION is poor for the affected eye, even when glucocorticoid treatment is started immediately. However, early recognition of AION and prompt aggressive treatment with high-dose glucocorticoids plus cyclophosphamide can prevent visual loss in the unaffected eye.

  13. Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.

    Science.gov (United States)

    Sada, Ken-Ei; Harigai, Masayoshi; Amano, Koichi; Atsumi, Tatsuya; Fujimoto, Shouichi; Yuzawa, Yukio; Takasaki, Yoshinari; Banno, Shogo; Sugihara, Takahiko; Kobayashi, Masaki; Usui, Joichi; Yamagata, Kunihiro; Homma, Sakae; Dobashi, Hiroaki; Tsuboi, Naotake; Ishizu, Akihiro; Sugiyama, Hitoshi; Okada, Yasunori; Arimura, Yoshihiro; Matsuo, Seiichi; Makino, Hirofumi

    2016-09-01

    To compare disease severity classification systems for six-month outcome prediction in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Patients with newly diagnosed AAV from 53 tertiary institutions were enrolled. Six-month remission, overall survival, and end-stage renal disease (ESRD)-free survival were evaluated. According to the European Vasculitis Study Group (EUVAS)-defined disease severity, the 321 enrolled patients were classified as follows: 14, localized; 71, early systemic; 170, generalized; and 66, severe disease. According to the rapidly progressive glomerulonephritis (RPGN) clinical grading system, the patients were divided as follows: 60, grade I; 178, grade II; 66, grade III; and 12, grade IV. According to the Five-Factor Score (FFS) 2009, 103, 109, and 109 patients had ≤1, 2, and ≥3 points, respectively. No significant difference in remission rates was found in any severity classification. The overall and ESRD-free survival rates significantly differed between grades I/II, III, and IV, regardless of renal involvement. Severe disease was a good predictor of six-month overall and ESRD-free survival. The FFS 2009 was useful to predict six-month ESRD-free survival but not overall survival. The RPGN grading system was more useful to predict six-month overall and ESRD-free survival than the EUVAS-defined severity or FFS 2009.

  14. Autoimmunity-Basics and link with periodontal disease.

    Science.gov (United States)

    Kaur, Gagandeep; Mohindra, Kanika; Singla, Shifali

    2017-01-01

    Autoimmune reactions reflect an imbalance between effector and regulatory immune responses, typically develop through stages of initiation and propagation, and often show phases of resolution (indicated by clinical remissions) and exacerbations (indicated by symptomatic flares). The fundamental underlying mechanism of autoimmunity is defective elimination and/or control of self-reactive lymphocytes. Periodontal diseases are characterized by inflammatory conditions that directly affect teeth-supporting structures, which are the major cause of tooth loss. Several studies have demonstrated the involvement of autoimmune responses in periodontal disease. Evidence of involvement of immunopathology has been reported in periodontal disease. Bacteria in the dental plaque induce antibody formation. Autoreactive T-cells, natural killer cells, ANCA, heat shock proteins, autoantibodies, and genetic factors are reported to have an important role in the autoimmune component of periodontal disease. The present review describes the involvement of autoimmune responses in periodontal diseases and also the mechanisms underlying these responses. Copyright © 2016 Elsevier B.V. All rights reserved.

  15. Development of measurement and analysis method for long-term monitoring of {sup 41}-K

    Energy Technology Data Exchange (ETDEWEB)

    Yuita, Koichi; Miyagawa, Saburo [National Inst. of Agro-Environmental Sciences, Tsukuba, Ibaraki (Japan)

    2000-02-01

    This study aimed to develop a double labeling method with {sup 41}K and {sup 15}N for animal feed and excreta. Guinea pig was used as the subjects for the preliminary experiment. Animal feces and urine were separately collected once a day and the feces were dried at 70degC and urine was lyophilized. Those samples were submitted to analysis after mixing. Then, {sup 41}KCl solution and {sup 15}NH{sub 4}SO{sub 4} solution were absorbed to the conventional guinea pig feed and 1.0 g of the feed was given once a day. The amount of {sup 41}K in feces was determined using flame photometric detector and {sup 15}N was determined by ANCA-SL Mass spectrometer. The isotope abundances of {sup 41}K and {sup 15}N in the feed were 6.11% and 0.829%, respectively and the excess % was -0.062 % and 0.46 % for {sup 41}K and {sup 15}N, respectively. The present results showed that 15-N labeling for feces was fairly succeeded, but {sup 41}K labeling was insufficient. Therefore, it is thought necessary to use K tracer of a larger excess % (-0.3% or more) and raise the accuracy of analysis for total K and {sup 41}K. (M.N.)

  16. Hepatic Sarcodosis presenting as portal hypertension in a young boy

    Science.gov (United States)

    Achakzai, Inamullah Khan; Majid, Zain; Khalid, Muhammad Ali; Khan, Shoaib Ahmed; Laeeq, Syed Mudassir; Luck, Nasir Hassan

    2018-01-01

    A 13-year-old boy, known case renal stone disease came with the complaints of abdominal pain along with low grade fever. On examination, hepatosplenomegaly was noted while his lab reports showed a low hemoglobulin with a raised ESR. His blood and urine cultures showed no growth. Viral markers, autoimmune profile, C and p ANCA were all negative apart from a raised serum IgG level. Ultrasound abdomen showed a hyperechoic liver with an enlarged spleen along with splenic varices and minimum ascites. Ultrasound hepatic doppler was normal. Serum AFP levels were normal while workup for Wilson’s disease was negative. Fibroscan showed F4 fibosis. CT scan abdomen showed an enlarged left lobe of the liver along with an enlarged spleen. His EGD revealed varices. So liver biopsy was done that was suggestive of chronic granulomatous disease with ZN stain testing negative for TB.PPD, urine for AFB were both negative. Serum ACE levels were raised. He started ATT therapy but his condition did not improve. So, on the suspicion of hepatic sarcoidosis, he started on steroids and had a drastic improvement in his condition. PMID:29564070

  17. Hepatic Sarcodosis presenting as portal hypertension in a young boy.

    Science.gov (United States)

    Achakzai, Inamullah Khan; Majid, Zain; Khalid, Muhammad Ali; Khan, Shoaib Ahmed; Laeeq, Syed Mudassir; Luck, Nasir Hassan

    2018-01-01

    A 13-year-old boy, known case renal stone disease came with the complaints of abdominal pain along with low grade fever. On examination, hepatosplenomegaly was noted while his lab reports showed a low hemoglobulin with a raised ESR. His blood and urine cultures showed no growth. Viral markers, autoimmune profile, C and p ANCA were all negative apart from a raised serum IgG level. Ultrasound abdomen showed a hyperechoic liver with an enlarged spleen along with splenic varices and minimum ascites. Ultrasound hepatic doppler was normal. Serum AFP levels were normal while workup for Wilson's disease was negative. Fibroscan showed F4 fibosis. CT scan abdomen showed an enlarged left lobe of the liver along with an enlarged spleen. His EGD revealed varices. So liver biopsy was done that was suggestive of chronic granulomatous disease with ZN stain testing negative for TB.PPD, urine for AFB were both negative. Serum ACE levels were raised. He started ATT therapy but his condition did not improve. So, on the suspicion of hepatic sarcoidosis, he started on steroids and had a drastic improvement in his condition.

  18. Microscopic polyangiitis: Atypical presentation with extensive small bowel necrosis, diffuse alveolar hemorrhage, and renal failure

    Directory of Open Access Journals (Sweden)

    Justin M. Segraves, M.D.

    2017-01-01

    Full Text Available Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO and perinuclear antineutrophil cytoplasmic (p-ANCA antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes. Further investigations after transfer to our facility determined that the patient was actually suffering from a severe exacerbation of previously diagnosed microscopic polyangiitis. He quickly developed diffuse alveolar hemorrhage (DAH necessitating intubation and acute kidney injury (AKI requiring dialysis. He subsequently developed mesenteric ischemia and bowel necrosis resulting in emergent laparotomy and extensive small bowel resection. Physicians need to be aware that microscopic polyangiitis can very rarely present with severe involvement of the abdominal viscera and mesenteric vessels. Severe disease necessitates the use of high dose IV steroids, rituximab or cyclophosphamide, and plasma exchange (PLEX.

  19. Microscopic polyangiitis: Atypical presentation with extensive small bowel necrosis, diffuse alveolar hemorrhage, and renal failure.

    Science.gov (United States)

    Segraves, Justin M; Iyer, Vivek N

    2017-01-01

    Microscopic polyangiitis is an uncommon systemic vasculitis of varying severity that is associated with myeloperoxidase (MPO) and perinuclear antineutrophil cytoplasmic (p-ANCA) antibodies. The most commonly affected organs are the lungs and kidneys. We report on a very unusual case of microscopic polyangiitis presenting with severe mesenteric ischemia in addition to diffuse alveolar hemorrhage and acute renal failure. The patient was initially diagnosed with acute pancreatitis at an outside facility given his severe abdominal pain and elevated pancreatic enzymes. Further investigations after transfer to our facility determined that the patient was actually suffering from a severe exacerbation of previously diagnosed microscopic polyangiitis. He quickly developed diffuse alveolar hemorrhage (DAH) necessitating intubation and acute kidney injury (AKI) requiring dialysis. He subsequently developed mesenteric ischemia and bowel necrosis resulting in emergent laparotomy and extensive small bowel resection. Physicians need to be aware that microscopic polyangiitis can very rarely present with severe involvement of the abdominal viscera and mesenteric vessels. Severe disease necessitates the use of high dose IV steroids, rituximab or cyclophosphamide, and plasma exchange (PLEX).

  20. Renal vasculitis presenting with acute kidney injury.

    Science.gov (United States)

    Villacorta, Javier; Diaz-Crespo, Francisco; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; Praga, Manuel; Fernandez-Juarez, Gema

    2017-06-01

    Renal failure secondary to ANCA-associated vasculitis represents a clinical and therapeutic challenge. In this study, we aimed to assess the treatment response rates and long-term outcomes of vasculitis patients presenting with renal failure. This retrospective study included 151 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Patients with renal failure which required dialysis at the onset were compared to those presenting with more preserved renal function. The primary end point was treatment response and patient surivival. Patients with severe renal involvement had a lower response to treatment compared to those having preserved renal function (26.6 versus 93.4%; p renal recovery (41.6 versus 12.5%; p = 0.05). A higher incidence of severe infections was observed among patients with severe renal involvement (38.4 versus 18.1%, p = 0.01). The mortality rate was significantly higher among vasculitis patients presenting with renal failure (53.8 versus 22.2%, p = 0.001). Global survival at 1 and 5 years was 60 and 47% in patients requiring dialysis compared with 90 and 80% among those with more preserved renal function (p renal dysfunction represents an independent risk factor for patient survival in renal vasculitis. Patients requiring dialysis associate a lower response rate to immunosuppressive therapy and a higher incidence of severe infections.

  1. Comparison of enzyme-linked immunosorbent assay and rapid chemiluminescent analyser in the detection of myeloperoxidase and proteinase 3 autoantibodies.

    Science.gov (United States)

    Pucar, Phillippa A; Hawkins, Carolyn A; Randall, Katrina L; Li, Candice; McNaughton, Euan; Cook, Matthew C

    2017-06-01

    Antibodies to myeloperoxidase (MPO) and proteinase 3 (PR3) are vital in the diagnosis and management of ANCA-associated vasculitis. A chemiluminescent immunoassay (CLIA; Quanta Flash) provides MPO and PR3 antibody results in 30 minutes, which is much faster than enzyme-linked immunosorbent assay (ELISA). We compared the performance of ELISA (Orgentec) and CLIA (Quanta Flash) for MPO and PR3 antibody quantitation on 303 samples, comprising 196 consecutive samples received in a single diagnostic laboratory over a 3 month period, and 107 samples collected from 42 known vasculitis patients over a 40 month period. We observed a correlation between both methods using spearman correlation coefficients (MPO, r s  = 0.63, p assays) and disease relapse (correlation for both MPO and PR3 antibody quantitation r s  = 0.84, p = 0.03 and r s  = 0.78, p ELISA for measurement of MPO and PR3 antibodies. Copyright © 2017. Published by Elsevier B.V.

  2. Autoimmune liver disease 2007.

    Science.gov (United States)

    Muratori, Paolo; Granito, Alessandro; Pappas, Georgios; Muratori, Luigi; Lenzi, Marco; Bianchi, Francesco B

    2008-01-01

    Autoimmune liver disease (ALD) includes a spectrum of diseases which comprises both cholestatic and hepatitic forms: autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and the so called "overlap" syndromes where hepatitic and cholestatic damage coexists. All these diseases are characterized by an extremely high heterogeneity of presentation, varying from asymptomatic, acute (as in a subset of AIH) or chronic (with aspecific symptoms such as fatigue and myalgia in AIH or fatigue and pruritus in PBC and PSC). The detection and characterization of non organ specific autoantibodies plays a major role in the diagnostic approach of autoimmune liver disease; anti nuclear reactivities (ANA) and anti smooth muscle antibodies (SMA) mark type 1 AIH, liver kidney microsomal antibody type 1 (LKM1) and liver cytosol type 1 (LC1) are the serological markers of type 2 AIH; antimitochondrial antibodies (AMA) are associated with PBC, while no specific marker is found in PSC, since anticytoplasmic neutrophil antibodies with perinuclear pattern (atypical p-ANCA or p-ANNA) are also detected in a substantial proportion of type 1 AIH cases. Treatment options rely on immunosoppressive therapy (steroids and azathioprine) in AIH and on ursodeoxycholic acid in cholestatic conditions; in all these diseases liver transplantation remains the only therapeutical approach for the end stage of liver disease.

  3. Autoantibodies and their antigens in autoimmune hepatitis.

    Science.gov (United States)

    Bogdanos, Dimitrios P; Mieli-Vergani, Giorgina; Vergani, Diego

    2009-08-01

    Autoantibody detection assists in the diagnosis and allows differentiation of autoimmune hepatitis (AIH) type 1 (AIH-1), characterized by antinuclear antibody (ANA) and/or smooth muscle antibody (SMA), and type 2 (AIH-2), distinguished by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) and/or antibodies to liver cytosol type 1 (anti-LC1). Detection of atypical perinuclear antineutrophil cytoplasmic antibodies (pANCA) and anti-soluble liver antigen (SLA) antibodies can act as an additional pointer toward the diagnosis of AIH, particularly in the absence of the conventional autoantibodies. Routine autoantibody testing by indirect immunofluorescence has been recently complemented by molecular assays based on purified or recombinant antigens. Although the AIH-1-specific ANA and SMA targets need better definition, those of anti-LKM1 and anti-LC1 in AIH-2 have been clearly identified; the fine specificity of antibody reactivity and its clinical relevance to disease pathogenesis are the focus of ongoing investigation. This article critically discusses the current knowledge of the diagnostic and clinical significance of AIH-related autoantibody reactivities, focusing on key issues that the physician needs to be aware of to be able to request the appropriate testing and to interpret correctly the laboratory results within the clinical context of the patient. Copyright Thieme Medical Publishers.

  4. Acute Kidney Injury and Atypical Features during Pediatric Poststreptococcal Glomerulonephritis

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    Rose M. Ayoob

    2016-01-01

    Full Text Available The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males, mean age of 8 years, were analyzed. Ninety-four percent had elevated serum BUN levels and decreased GFR. Four of the hospitalized patients had complex presentations that included AKI along with positive ANA or ANCAs. Three patients required renal replacement therapy and two were thrombocytopenic. PSGN usually does not occur as a severe nephritis. Over the 12-month study period, 17 cases associated with low serum albumin in 53%, acute kidney injury in 94%, and thrombocytopenia in 18% were treated. The presentation of PSGN may be severe and in a small subset have associations similar to SLE nephritis findings including AKI, positive ANA, and hematological anomalies.

  5. EUROPattern Suite technology for computer-aided immunofluorescence microscopy in autoantibody diagnostics.

    Science.gov (United States)

    Krause, C; Ens, K; Fechner, K; Voigt, J; Fraune, J; Rohwäder, E; Hahn, M; Danckwardt, M; Feirer, C; Barth, E; Martinetz, T; Stöcker, W

    2015-04-01

    Antinuclear autoantibodies (ANA) are highly informative biomarkers in autoimmune diagnostics. The increasing demand for effective test systems, however, has led to the development of a confusingly large variety of different platforms. One of them, the indirect immunofluorescence (IIF), is regarded as the common gold standard for ANA screening, as described in a position statement by the American College of Rheumatology in 2009. Technological solutions have been developed aimed at standardization and automation of IIF to overcome methodological limitations and subjective bias in IIF interpretation. In this review, we present the EUROPattern Suite, a system for computer-aided immunofluorescence microscopy (CAIFM) including automated acquisition of digital images and evaluation of IIF results. The system was originally designed for ANA diagnostics on human epithelial cells, but its applications have been extended with the latest system update version 1.5 to the analysis of antineutrophil cytoplasmic antibodies (ANCA) and anti-dsDNA antibodies. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  6. Controversies on Rituximab Therapy in Sjögren Syndrome-Associated Lymphoproliferation

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    Luca Quartuccio

    2009-01-01

    Full Text Available Sjögren's syndrome (SS is a systemic autoimmune disease characterized by chronic inflammation of salivary and lachrymal glands, and frequently accompanied by systemic symptoms. A subgroup of SS patients develops malignant B cell non-Hodgkin's lymphoma (NHL, usually of the mucosa-associated lymphoid tissue (MALT type and very often located in the major salivary glands. Currently, there is a lack of evidence-based intervention therapy which may influence SS-related chronic inflammation and lymphoproliferation. B cells are involved in the pathogenesis of SS, and B cell downregulation may lead to a decrease of disease activity. Rituximab (RTX, a chimeric monoclonal antibody targeting the CD20 antigen on the B cell surface, has been successfully investigated in other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, ANCA-associated vasculitis, and mixed cryoglobulinemic syndrome. Preliminary experiences of RTX therapy in SS patients with or without a lymphoproliferative disorder suggest that SS patients with more residual exocrine gland function might better benefit from RTX. Efficacy of RTX in SS-associated B-cell lymphoma, mainly in low-grade salivary gland lymphomas, remains an open issue.

  7. Science as drama

    Energy Technology Data Exchange (ETDEWEB)

    Crease, R.P. [Department of Philosophy, State University of New York, Stony Brook (United States) and Brookhaven National Laboratory (United States)]. E-mail: rcrease@notes.cc.sunysb.edu

    2006-09-15

    Ludwig Boltzmann, who died 100 years ago this month, played a critical role in the development of thermodynamics. However, he was just one of a colourful cast of characters in a hugely dramatic story, says Robert P Crease. Lazare Carnot (1753-1823), a military engineer whose particular talent is uncovering and eliminating administrative and mechanical inefficiency, publishes a treatise on water-powered machines. Follow the water, he writes: the maximum power depends on how great a distance it falls. Track down and eradicate sources of waste to make your machine work better. Count Rumford (1753-1814), soldier of fortune and amateur scientist, is in Munich. Keen to reveal the mysteries of heat, he puts a six-pound brass cannon in a vat of water, inserts a drill bit driven by a winch, hitches up a horse to the winch, and finds that enough heat has been generated through the drilling to boil the water in two-and-a-half hours. He concludes that the caloric theory- formulated by Antoine Lavoisier, a former husband of one of Rumford's mistresses - is wrong, because the heat is obviously a form of motion coming from the friction between the bit and the cannon. Sadi Carnot (1796-1832), a quiet engineer, returns from his father Lazare's deathbed. Determined to carry on his father's work, he composes Reflections on the Motive Power of Heat. Follow the heat, he writes. Caloric in a heat engine, like water in a water engine, is conserved as it flows from hot to cold, and the maximum power depends on the magnitude of the temperature drop. The most efficient machine is modelled by an ideal cycle of expansion and compression in which the engine works reversibly, the caloric being conserved in going back and forth between the two temperature endpoints with no heat loss due to friction or dissipation. This is a key insight, but Reflections is almost totally ignored. He publishes nothing more, then catches scarlet fever, brain fever and cholera, before dying, aged 36

  8. Realistic modeling of seismic input for megacities and large urban areas

    Science.gov (United States)

    Panza, G. F.; Unesco/Iugs/Igcp Project 414 Team

    2003-04-01

    , supply a particularly powerful tool for the prevention aspects of Civil Defense. We present a selection of the main results obtained for the cities of Algiers, Beijing, Bucharest, Cairo, Debrecen, Delhi, Naples, Rome, Russe, Santiago de Cuba, Sofia, Thessaloniki and Zagreb. The UNESCO/IUGS/IGCP PROJECT 414 team members are: Giuliano F. Panza (1,2) (Chairman), Leonardo Alvarez (3), Abdelkrim Aoudia (1,2), Abdelhakim Ayadi (4), Hadj Benhallou (4,5), Djillali Benouar (6), Zoltan Bus (7), Yun-Tai Chen (8), Carmen Cioflan (9), Zhifeng Ding (8), Attia El-Sayed (10), Julio Garcia (3), Bartolomeo Garofalo (11), Alexander Gorshkov (12), Katalin Gribovszki (13), Assia Harbi (4), Panagiotis Hatzidimitriou (14), Marijan Herak (15), Mihaela Kouteva (16), Igor Kuznetzov (12), Ivan Lokmer (15), Said Maouche (4), Gheorghe Marmureanu (9), Margarita Matova (16), Maddalena Natale (11), Concettina Nunziata (11), Imtiyaz Parvez (17,1), Ivanka Paskaleva (16), Ramon Pico (18), Mircea Radulian (9), Fabio Romanelli (2), Alexander Soloviev (12), Peter Suhadolc (2), Gyõzõ Szeidovitz (7), Petros Triantafyllidis (14), Franco Vaccari (2,19). (1) The Abdus Salam International Center for Theoretical Physics, SAND Group, Mirarmar, Trieste, Italy. (2) Department of Earth Sciences, University of Trieste, Via E. Weiss 1, 34127 Trieste, Italy. (3) Centro Nacional de Investigaciones Sismologicas, Cuba. (4) Centre de Recherche en Astronomie, Astrophysique et Geophysique, BP. 63, Bouzaréah, Alger, Algérie. (5) Faculté des Sciences de la Terre de l'Aménagment du Territoire et de la Géographie, USTHB, Alger, Algérie. (6) University of Algiers (USTHB), Civil Engineering Dpt., Alger, Algeria. (7) Hungarian Academy of Sciences, Seismological Observatory of GGRI, H-1112 Budapest, Meredek u. 18, Hungary. (8) Institute of Geophysics, China Seismological Bureau, Beijing, 100081, China. (9) National Institute for Earth Physics, Calugareni 12, P.O.Box: MG 2, 76900 Bucharest-Magurele, Romania. (10) Department of

  9. Mastoidite e paralisia facial como manifestações iniciais de Granulomatose de Wegener Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    André Souza de Albuquerque Maranhão

    2012-04-01

    Full Text Available A Granulomatose de Wegener (GW é caracterizada por granulomas necrotizantes e vasculite. Sem tratamento a doença tem prognóstico pobre com índice de mortalidade de 90% em 2 anos. Diversos autores citam as manifestações otológicas no curso da GW, entretanto não é especificado em que momento da doença elas apareceram, isto é, se como manifestação inicial ou subsequente a outros achados. Objetivo: Descrever três casos confirmados de GW que apresentaram inicialmente mastoidite e evoluíram com paralisia facial periférica (PFP. Material e Método: Estudo de série de casos. Pacientes diagnosticados com GW que apresentaram inicialmente manifestações otológicas são descritos. Resultados: Os três casos descritos abriram o quadro com otalgia, otorreia e hipoacusia unilateral, associada a paralisia facial periférica ipsilateral. Tiveram resposta inadequada aos tratamentos instituídos o que motivou uma investigação de outras doenças associadas. Nessas circunstâncias, detectaram-se títulos positivos de ANCA-C em todos pacientes, confirmando-se o diagnóstico de GW, após período variável de investigação. Institui-se o tratamento para GW observando-se melhora do quadro clínico, regressão da PFP e melhora parcial dos limiares auditivos. Conclusão: Complicações de otites médias agudas (mastoidite e PFP refratárias as terapêuticas habituais impõem a investigação de doenças associadas e a GW deverá ser pesquisada para que se possa fazer o diagnóstico o mais precocemente possível, alterando desta forma o prognóstico destes pacientes.Wegener's Granulomatosis (WG is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor - a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present - whether as initial manifestations or subsequent to

  10. Características físico-mecânicas do couro de caprinos mestiços Boer e Anglo Nubianos Physical-mechanic characteristics of leather from crossbred Boer and Anglo Nubiano goats

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    Arturo Bernardo Selaive Villarroel

    2004-12-01

    Full Text Available As características físico-mecânicas de 22 couros de caprinos mestiços Boer e Anglo Nubianos foram avaliadas para determinação da qualidade quanto à resistência à tração e ao rasgamento. Os animais foram abatidos com idade média de 260 dias e peso corporal de 22,0 kg. Após a esfola, as peles foram pesadas, conservadas por salmouragem e salga e curtidas com sulfato de cromo. Para determinação da resistência à tração e ao rasgamento progressivo, foram retirados das regiões paleta, anca e ventre dos couros semi-acabados seis corpos-de-prova nas posições paralela e perpendicular à linha dorsal. O delineamento experimental utilizado foi o de parcelas subdivididas, tendo como parcelas dois grupos genéticos e como subparcelas o fatorial 2 x 3 (duas posições e três regiões, com três repetições, sendo as médias comparadas pelo teste Tukey. Não houve diferenças significativas de resistência entre os couros de ambos os grupos genéticos para os ensaios de tração e de rasgamento progressivo. Entretanto, observaram-se diferenças significativas entre as regiões paleta, anca e ventre em todos os parâmetros estudados nos ensaios de tração e de rasgamento progressivo, com os maiores valores de espessura, resistência e alongamento encontrados na paleta. Concluiu-se que o uso de reprodutores exóticos da raça Boer ou Anglo Nubiano em cruzamentos, para melhorar a produção de carne dos caprinos da região nordeste, não influencia a qualidade do couro dos animais.The physical-mechanic industry characteristics of 22 crossed goat leathers from native does mated with Anglo Nubian (AG and Boer (BO bucks were studied to evaluate the leather quality by analysis of tension and tear resistance. Animals were slaughtered at average of 260 days of age and 22.0 kg of live body weigth. After skinning, the pelts were weighed, conserved in brine and salted and classified by quality before the chromium-tanned. To evaluate the tension and

  11. KARAKTERISTIK HABITAT DAN MORFOLOGI SIPUT ONGCOMELANIA HUPENSIS LINDOENSIS SEBAGAI HEWAN RESERVOIR DALAM PENULARAN SHISTOSOMIASIS PADA MANUSIA DAN TERNAK DI TAMAN NASIONAL LORE LINDU (Habitat Characteristics and Morphology of Oncomelania hupensis

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    Hafsah Hafsah

    2013-07-01

    Full Text Available ABSTRAK Penelitian ini bertujuan untuk mengkaji habitat dan morfologi siput Oncomelania hupensis lindoensis sebagai hewan reservoir dalam penularan shistosomiasis pada manusia dan ternak. Penelitian dilakukan dalam kawasan Taman Nasional Lore Lindu. Metode yang digunakan adalah metode survei dengan mengukur dan mengambil beberapa sampel tanah pada beberapa jenis habitat. Siput dikoleksi dengan menggunakan metode gelang besi yang disebut ring method. Siput yang dikumpulkan kemudian dibawa ke laboratorium untuk pengamatan bentuk morfologi dan mirasidia baik secara langsung maupun dengan penggunaan mikroskop. Penentuan tingkat prevalensi digunakan metode “ Kato-Kars” yang dimodifikasi. Data dianalisis secara deskriptif berdasarkan data hasil survei di lapangan dan hasil analisis dari laboratorium. Hasil penelitian menunjukkan bahwa habitat siput O.hupensis Lindoensis yang terdapat dalam kawasan Taman Nasional Lore Lindu sebanyak 144 habitat (fokus dan terdistribusi pada empat desa yaitu Tomado (64 fokus, Anca (63 fokus, Puroo (11 fokus dan Langko (6 fokus dengan persebaran 44,44 % ( sawah, 29,86 % ( kebun, 18,06 % ( padang rumput, dan 11 % ( hutan. Karakteristik habitat yaitu tekstur tanah lempung berpasir dengan bahan organik tanah yang relatif rendah (2%. Pada ternak didapatkan tingkat prevalensi yaitu kerbau (39,36%, sapi (39,32%, dan babi (22,5%. Kesimpulan dari hasil penelitian ini bahwa habitat siput O. Hupensis lindoensis mempunyai karakteristik dan bentuk yang spesifik. Tingkat prevalensi schistosomiasis pada manusia dan ternak dalam kawasan Taman Nasional Lore Lindu masih cukup tinggi. ABSTRACT The objective of the study was evaluated the habitat characteristics and  morphology of  Oncomelania hupensis lindoensis  as  a  reservoir in transmission of Schistosomiasis on  human and animal in Lore Lindu National Park. The study was conducted in four villages as known as the habitat of the endemic snails. Collections of the snails

  12. [Glomerulonephritis and vasculitis as causes of arterial hypertension].

    Science.gov (United States)

    Eicken, Sibylle; Gugger, Mathias; Marti, Hans-Peter

    2012-05-01

    , such as a screening for infections, including search for HIV, hepatitis B or C and various bacteria, and for systemic inflammatory diseases, including tests for antibodies, such as ANA, anti-dsDNA, ANCA, anti-GBM and anti-CCP. In cases of membranous nephropathy, antibodies against phospholipase-A2-receptor need to be looked for. Depending upon the given clinical circumstances and the type of disease, a reasonable tumor screening must be performed, especially in cases of membranous and minimal-change nephropathy. Finally, radiological examinations will complete the initial work-up. In most cases, at least an ultrasound of the kidney is mandatory. Thereafter, in most cases a renal biopsy is required to establish a firm diagnosis to define all treatment options and their chance of success. The elimination of a specific cause for a given glomerulonephritis or vasculitis, such as an infection, a malignancy or a drug-related side-effect, remains the key principle in the management of these diseases. ACE-inhibitors, angiotensin receptor-blockers, aldosteron antagonists and renin-inhibitors remain the mainstay in the therapy of arterial hypertension with proteinuria. Only in cases of persistently high proteinuria, ACE-inhibitors and angiotensin receptor blockers can be prescribed in combination. Certain types of glomerulonephritis and essentially all forms of vasculitis require some form of more specific anti-inflammatory therapy. Respective immunosuppressive drug regimens contain traditionally medications, such as glucocorticoids (e. g. prednisone), cyclosporine A, mycophenolate mofetil, cyclophosphamide, and azathioprine. With respect to more severe forms of glomerulonephritis and vasculitis, the antibody rituximab represents a new and less toxic alternative to cyclophosphamide. Finally, in certain special cases, like Goodpasture's syndrome or severe ANCA-positive vasculitis, a plasma exchange will be useful and even required.

  13. Características generales de 29 pacientes con vasculitis de pequeños vasos General characteristics of 29 patients with small vessel vasculitis

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    Nicolás Di Benedetto

    2010-04-01

    Full Text Available El objetivo de esta serie fue describir las características generales y manifestaciones clínicas de pacientes con vasculitis de pequeños vasos que concurrieron al servicio de autoinmunidad de un hospital de la comunidad y compararlas con la literatura. Se revisaron de manera retrospectiva las historias clínicas archivadas bajo el rótulo de vasculitis en un período de 16 años. Se seleccionaron aquellos pacientes que cumplieran criterios diagnósticos de vasculitis de pequeños vasos. Se extrajeron los datos de interés y se constató la supervivencia mediante contacto telefónico. Posteriormente se realizó una búsqueda bibliográfica y se compararon los resultados. Se incluyeron 13 pacientes con granulomatosis de Wegener, 6 con síndrome de Churg Strauss y 10 con poliangeítis microscópica. El 55% (16 fueron menores de 55 años al diagnóstico y hubo predominio del sexo femenino (2.6:1. La demora diagnóstica fue mayor a un año en el 46% de los casos. Los sistemas más frecuentemente afectados fueron respiratorio y otorrinolaringológico. El 79% presentó anticuerpos anti-citoplasma de neutrófilos (ANCA positivos. La mortalidad fue del 24% (7/29. Al comparar las características de los pacientes incluidos en esta serie con lo descripto en la literatura, se hallaron diferencias en cuanto a la forma de presentación, sistemas comprometidos y porcentaje de pacientes ANCA positivos; además se observó que el resultado negativo de este marcador parece asociarse con mayor demora diagnóstica y en consecuencia peor pronóstico, lo que remarca su importancia como herramienta adicional no invasiva al momento del diagnóstico.The objective of this series was to describe the general characteristics and clinical manifestations of patients with small vessel vasculitis who were assisted in the autoimmunity department of a community public hospital and to compare the results with the literature. Clinical records under the label of vasculitis in a

  14. Kidney involvement in rheumatoid arthritis

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    P. Lazzarini

    2011-09-01

    Full Text Available Rheumatoid Arthritis (RA is a widespread disease and its renal involvement, relatively common, is clinically significant because worsens course and mortality of the primary disease. There is still no agreement on the prevalence of renal disorders in RA: data analysis originates from different sources, as death certificates, autopsies, clinical and laboratory findings and kidney biopsies, each with its limitations. Histoimmunological studies on bioptical specimens of patients with RA and kidney damage, led to clarify prevalent pathologies. In order of frequency: glomerulonephritis and amyloidosis (60-65% and 20-30% respectively, followed by acute or chronic interstitial nephritis. Kidney injury during RA includes secondary renal amyloidosis, nephrotoxic effects of antirheumatic drugs and nephropathies as extra-articular manifestations (rheumatoid nephropathy. Amyloidosis affects survival, increases morbidity and is the main cause of end stage renal disease in patients with RA and nephropathy. Strong association between RA activity and amyloidosis needs the use of immunosuppressive and combined therapies, to prevent this complication and reduce risk of dialysis. Long-lasting and combined RA pharmacotherapy involves various renal side effects. In this review we describe NSAIDs and DMARDs (Disease-Modifying Antirheumatic Drugs nephrotoxicity, particularly by gold compounds, D-penicillamine, cyclosporine A and methotrexate. Rare cases of IgA glomerulonephritis during immunomodulating therapy with leflunomide and TNF blocking receptor (etanercept are reported; real clinical significance of this drug-related nephropathy will be established by development of RA treatment. In RA nephropathies, mesangial glomerulonephritis is the most frequent histological lesion (35-60 % out of biopsies from patients with urinary abnormalities and/or kidney impairment, followed by minimal change glomerulopathy (3-14% and p-ANCA positive necrotizing crescentic

  15. Autoantibodies other than anti-desmogleins in pemphigus vulgaris patients

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    Marwah Adly Saleh

    2017-01-01

    Full Text Available Background: Pemphigus vulgaris (PV is an immunoglobulin G-mediated autoimmune bullous skin disease. Nonorgan-specific antibodies were detected in Tunisian and Brazilian pemphigus patients with different prevalence. Materials and Methods: Fifty PV patients and fifty controls were screened for antinuclear antibodies (ANAs, anti-smooth muscle antibodies (ASMAs, anti-parietal antibodies (APAs, anti-mitochondrial antibodies, and Anti-nuclear cytoplasmic antibodies (ANCA by indirect immunofluorescence. Results: Thirty-nine patients were female and 11 were male. Fifteen patients did not receive treatment before while 35 patients were on systemic steroid treatment ± azathioprine. Twenty (40% of the PV patients and 1 (2% control had positive ANA. ANA was significantly higher in PV patients than controls, P< 0.0001. ASMAs were detected in 20 (40% PV patients and none of the controls. ASMA was significantly higher in PV patients than controls, P< 0.0001. No significant difference was detected between treated and untreated regarding ANA, P - 0.11. However, there was a significant difference between treated and untreated regarding ASMA, P- 0.03. Six patients (12% and none of the controls had positive APA. There was a significant difference between the patients and the controls in APA. P- 0.027. Conclusion: Egyptian PV patients showed more prevalent ANA, ASMA, and APA than normal controls. Follow-up of those patients is essential to detect the early development of concomitant autoimmune disease. Environmental factors might account for the variability of the nonorgan-specific antibodies among different populations.

  16. Epigenetics and Vasculitis: a Comprehensive Review.

    Science.gov (United States)

    Renauer, Paul; Coit, Patrick; Sawalha, Amr H

    2016-06-01

    Vasculitides represent a group of relatively rare systemic inflammatory diseases of the blood vessels. Despite recent progress in understanding the genetic basis and the underlying pathogenic mechanisms in vasculitis, the etiology and pathogenesis of vasculitis remain incompletely understood. Epigenetic dysregulation plays an important role in immune-mediated diseases, and the contribution of epigenetic aberrancies in vasculitis is increasingly being recognized. Histone modifications in the PR3 and MPO gene loci might be mechanistically involved in the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Similarly, other studies revealed important epigenetic contribution to other vasculitides, including Kawasaki disease and IgA vasculitis. More recently, genome-wide epigenomic studies have been performed in several vasculitides. A recent genome-wide DNA methylation study uncovered an important role for epigenetic remodeling of cytoskeleton-related genes in the pathogenesis of Behçet's disease and suggested that reversal of some of these DNA methylation changes associates with disease remission. Genome-wide DNA methylation profiling characterized the inflammatory response in temporal artery tissue from patients with giant cell arteritis and showed increased activation of calcineurin/nuclear factor of activated T cells (NFAT) signaling, prompting the suggestion that a specific calcineurin/NFAT inhibitor that is well tolerated and with the added beneficial anti-platelet activity, such as dipyridamole, might be of therapeutic potential in giant cell arteritis. While epigenetic studies in systemic vasculitis are still in their infancy, currently available data clearly indicate that investigating the epigenetic mechanisms underlying these diseases will help to better understand the pathogenesis of vasculitis and provide novel targets for the development of disease biomarkers and new therapies.

  17. Exploration, Development, and Validation of Patient-reported Outcomes in Antineutrophil Cytoplasmic Antibody–associated Vasculitis Using the OMERACT Process

    Science.gov (United States)

    Robson, Joanna C.; Milman, Nataliya; Tomasson, Gunnar; Dawson, Jill; Cronholm, Peter F.; Kellom, Katherine; Shea, Judy; Ashdown, Susan; Boers, Maarten; Boonen, Annelies; Casey, George C.; Farrar, John T.; Gebhart, Don; Krischer, Jeffrey; Lanier, Georgia; McAlear, Carol A.; Peck, Jacqueline; Sreih, Antoine G.; Tugwell, Peter; Luqmani, Raashid A.; Merkel, Peter A.

    2016-01-01

    Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of linked multisystem life- and organ-threatening diseases. The Outcome Measures in Rheumatology (OMERACT) vasculitis working group has been at the forefront of outcome development in the field and has achieved OMERACT endorsement of a core set of outcomes for AAV. Patients with AAV report as important some manifestations of disease not routinely collected through physician-completed outcome tools; and they rate common manifestations differently from investigators. The core set includes the domain of patient-reported outcomes (PRO). However, PRO currently used in clinical trials of AAV do not fully characterize patients’ perspectives on their burden of disease. The OMERACT vasculitis working group is addressing the unmet needs for PRO in AAV. Methods Current activities of the working group include (1) evaluating the feasibility and construct validity of instruments within the PROMIS (Patient-Reported Outcome Measurement Information System) to record components of the disease experience among patients with AAV; (2) creating a disease-specific PRO measure for AAV; and (3) applying The International Classification of Functioning, Disability and Health to examine the scope of outcome measures used in AAV. Results The working group has developed a comprehensive research strategy, organized an investigative team, included patient research partners, obtained peer-reviewed funding, and is using a considerable research infrastructure to complete these interrelated projects to develop evidence-based validated outcome instruments that meet the OMERACT filter of truth, discrimination, and feasibility. Conclusion The OMERACT vasculitis working group is on schedule to achieve its goals of developing validated PRO for use in clinical trials of AAV. (First Release September 1 2015; J Rheumatol 2015;42:2204–9; doi:10.3899/jrheum.141143) PMID:26329344

  18. Vasculitis in patients with inflammatory bowel diseases: A study of 32 patients and systematic review of the literature.

    Science.gov (United States)

    Sy, Alice; Khalidi, Nader; Dehghan, Natasha; Barra, Lillian; Carette, Simon; Cuthbertson, David; Hoffman, Gary S; Koening, Curry L; Langford, Carol A; McAlear, Carol; Moreland, Larry; Monach, Paul A; Seo, Philip; Specks, Ulrich; Sreih, Antoine; Ytterberg, Steven R; Van Assche, Gert; Merkel, Peter A; Pagnoux, Christian

    2016-02-01

    Published small case series suggest that inflammatory bowel disease [IBD; Crohn's disease (CD) or ulcerative colitis (UC)] and vasculitis co-occur more frequently than would be expected by chance. To describe this association by an analysis of a large cohort of carefully studied patients and through a systematic literature review. Patients with both IBD and vasculitis enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers and/or in the University of Toronto's IBD clinic were included in this case series. A systematic literature review of patients with IBD and vasculitis involved a PubMed search through February 2014. The main characteristics of patients with Takayasu arteritis (TAK) and IBD were compared to those in patients with TAK without IBD followed in the VCRC. The study identified 32 patients with IBD and vasculitis: 13 with large-vessel vasculitis [LVV; 12 with TAK, 1 with giant cell arteritis (GCA); 8 with CD, 5 with UC]; 8 with ANCA-associated vasculitis [AAV; 6 granulomatosis with polyangiitis (GPA), 2 with eosinophilic granulomatosis with polyangiitis (EGPA)]; 5 with isolated cutaneous vasculitis; and 6 with other vasculitides. Patients with LVV and AAV were mostly female (18/21). The diagnosis of IBD preceded that of vasculitis in 12/13 patients with LVV and 8/8 patients with AAV. The review of the literature identified 306 patients with IBD and vasculitis: 144 with LVV (133 TAK; 87 with IBD preceding LVV), 19 with AAV [14 GPA, 1 EGPA, 4 microscopic polyangiitis (MPA)], 66 with isolated cutaneous vasculitis, and 77 with other vasculitides. Patients with IBD and TAK were younger and had more frequent headaches, constitutional symptoms, or gastrointestinal symptoms compared to those patients in the VCRC who had TAK without IBD. These findings highlight the risk of vasculitis, especially TAK, in patients with IBD (both CD and UC). Copyright © 2015 Elsevier

  19. Urinary Biomarkers in Relapsing Antineutrophil Cytoplasmic Antibody-associated Vasculitis

    Science.gov (United States)

    Lieberthal, Jason G.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.; Monach, Paul A.

    2015-01-01

    Objective Glomerulonephritis (GN) is common in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), but tools for early detection of renal involvement are imperfect. We investigated 4 urinary proteins as markers of active renal AAV: alpha-1 acid glycoprotein (AGP), kidney injury molecule-1 (KIM-1), monocyte chemoattractant protein-1 (MCP-1), and neutrophil gelatinase-associated lipocalin (NGAL). Methods Patients with active renal AAV (n = 20), active nonrenal AAV (n = 16), and AAV in longterm remission (n = 14) were identified within a longitudinal cohort. Urinary biomarker concentrations (by ELISA) were normalized for urine creatinine. Marker levels during active AAV were compared to baseline remission levels (from 1–4 visits) for each patient. Areas under receiver-operating characteristic curves (AUC), sensitivities, specificities, and likelihood ratios (LR) comparing disease states were calculated. Results Baseline biomarker levels varied among patients. All 4 markers increased during renal flares (p < 0.05). MCP-1 discriminated best between active renal disease and remission: a 1.3-fold increase in MCP-1 had 94% sensitivity and 89% specificity for active renal disease (AUC = 0.93, positive LR 8.5, negative LR 0.07). Increased MCP-1 also characterized 50% of apparently nonrenal flares. Change in AGP, KIM-1, or NGAL showed more modest ability to distinguish active renal disease from remission (AUC 0.71–0.75). Hematuria was noted in 83% of active renal episodes, but also 43% of nonrenal flares and 25% of remission samples. Conclusion Either urinary MCP-1 is not specific for GN in AAV, or it identifies early GN not detected by standard assessment and thus has potential to improve care. A followup study with kidney biopsy as the gold standard is needed. PMID:23547217

  20. Circulating C3 levels predict renal and global outcome in patients with renal vasculitis.

    Science.gov (United States)

    Villacorta, Javier; Diaz-Crespo, Francisco; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; Praga, Manuel; Fernandez-Juarez, Gema

    2016-11-01

    Several studies have demonstrated the crucial role of complement activation in the pathogenesis of ANCA-associated vasculitis. We aimed to assess the association between baseline serum C3 (sC3) levels and long-term outcomes in patients with renal vasculitis. This retrospective study included 111 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Serum levels of C3 were measured at the onset and the study population was divided into three tertiles according to sC3 concentrations (tertile 1 128 mg/dl). Patients with lower sC3 (tertile 1) were compared with those having higher levels of sC3 (tertile 2 and tertile 3). Histological, clinical, and laboratory data were recorded for analysis. The primary end point was the composite of end-stage renal disease (ESRD) and death from any cause. Lower sC3 levels were associated with a higher need for dialysis and lower response rate to treatment (p = 0.04 and p = 0.007, respectively). Renal and global survival at 1 and 5 years was 53 and 46 % in patients with lower sC3 (tertile 1) compared with 72 and 65 % in patients with higher sC3 (upper two tertiles) (p = 0.04). In a multivariate Cox-regression model, when adjusted by renal function and histopatholologic categories, lower sC3 remained as an independent predictor of ESRD and death (HR, 1.9; 95 % CI, 1.1 to 3.4; p = 0.02). Baseline serum C3 levels have an independent prognostic value in predicting long-term renal and global survival in patients with renal vasculitis.

  1. Globalizm ve Dini Değerlerdeki Dönüşüm

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    Şaban Ali Düzgün

    2015-11-01

    Full Text Available Globalisin is an emerging sensibility of our post-modern, post-industrial era. It is the determining characteristics of world affairs in an integrated global marketplace whose lingua if anca is the language of the Internet. Within this emerging power the world’s greater and lesser political entities (countries, city-states, religions and traditions now meet in the large, but also in the small of interpersonal acts and interactions in many regions and most cities of the world. Through developments in communication we now form an increasingly cosmopolitan population which meets frequently in actual interaction and in the global virtual reality. Within this context the world’s greatest gathering traditions and ideas are exchanging and changing. West meets East as South affects North. Western (Judeo-Christian-Islamic, Confucian, Buddhistic, Amerindian, African concepts express themselves in politics, in ecology, in economics, in intellectual exchanges and critiques which affect us in myriad ways. In some contexts these ideas expand and enlarge all our thinking. In others, they seem to conflict and compete as each of the large traditions. With its advantages and drawback this process is still pregnant for the future. It is beyond doubt that this process is mostly challenging for the traditional religions (not for New Age ones, as the space, time, unified people they were accustomed to addressing in the ongoing process to date are now under many attacks and they have to develop new means of protecting their adherens on the one hand and of developing them on the other

  2. Urinary levels of high mobility group box-1 are associated with disease activity in antineutrophil cytoplasmic autoantibody-associated vasculitis.

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    Tian-Tian Ma

    Full Text Available High mobility group box-1 (HMGB1, a kind of pro-inflammatory mediator, is associated with inflammatory conditions and tissue damage. Our previous study demonstrated that the circulating levels of HMGB1 correlated with disease activity of antineutrophil cytoplasmic antibody (ANCA-associated vasculitis (AAV. In the current study, we aimed to measure urinary levels of HMGB1 in AAV patients, correlated them to clinical activity index and analysed the immunohistochemical HMGB1 staining in kidney specimens.50 patients with AAV in active stage and 56 patients with AAV in remission were recruited. The urinary levels of HMGB1 were determined by enzyme-linked immunosorbent assay. Moreover, renal biopsy specimens from 27 patients with active AAV were randomly collected to evaluate the deposition of HMGB1.Urinary HMGB1 levels in AAV patients in active stage were significantly higher than those in AAV patients in remission and healthy controls (1.46 [0.56-3.43] versus 0.38 [0.10-1.35] mg/μmolCr, P=0.001; 1.46 [0.56-3.43] versus 0.48 [0.40-0.60] mg/μmolCr, P=0.000, respectively. Further analysis found that urinary levels of HMGB1 correlated with erythrocyte sedimentation rate (r=0.354, p=0.012, C-reactive protein (r=0.289, p=0.042, and Birmingham Vasculitis Activity Score (r=0.350, p=0.013. Renal tissue of active AAV patients showed HMGB1 was mainly expressed in the cytoplasm and the extracellular space. The percentage of HMGB1-negative nuclei in renal tissue of patients with active AAV was significantly higher than that in normal controls (60.6±20.2 % versus 2.7±0.6 %, p<0.01.Urinary levels of HMGB1 may be associated with the disease activity in AAV patients.

  3. A rare case of Addison's disease, hepatitis, thyreoiditis, positive IgG anti-tissue transglutaminase antibodies and partial IgA deficiency.

    Science.gov (United States)

    Baleva, Marta P; Mihaylova, Snejina; Yankova, Petja; Atanasova, Iliana; Nikolova-Vlahova, Milena; Naumova, Elissaveta

    2016-01-01

    Selective IgA deficiency (IgAD) is the most prevalent type of primary immune deficiencies, but partial IgA deficiency is even more common. Addison's disease is a rare condition associated with primary adrenal insufficiency due to infection or autoimmune destruction of the adrenals. The association between IgA deficiency and Addison's disease is very rare. We observed a 22-year-old male patient with marked darkening of the skin, especially on the palms and areolae, jaundice on the skin and sclera, astheno-adynamia, hypotension (80/50 mm Hg), and pain in the right hypochondrium. The laboratory investigations revealed increased serum levels of total and indirect bilirubin, AST, ALT, GGT and LDH, negative HBsAg, anti-HBc IgM, anti-HCV and anti-HAV IgM, very low serum IgA levels (0.16 g/l) with normal IgG and IgM, negative ANA, ANCA, AMA, LKM-1, anti-GAD-60, anti-IA-2, anti-thyroglobulin antibodies, a mild increase in anti-TPO antibodies titer, a marked increase in IgG anti-tissue transglutaminase antibodies, with no typical changes in cellular immunity, negative T-SPOT-TB test, HLA - A*01; B*08; DRB1*03; DQB1*02, karyotype - 46, XY. We present a rare case of partial IgA deficiency with Addison's disease, hepatitis, thyroiditis and positive anti-tissue transglutaminase antibodies. IgAD and some autoimmune disorders share several predisposing HLA genes, thus explaining the increased prevalence of IgAD in certain patient groups.

  4. Aqua ammonia 15 N obtaining and application with vainness for sugar-cane fertilization

    International Nuclear Information System (INIS)

    Vitti, Andre Cesar; Trivellin, Paulo Cesar O.; Oliveira, Claudineia R. de; Bendassoli, Jose A.

    2000-01-01

    Nitrogen compounds marked with the isotope 15 N are continuously being used in agronomic studies and, when associated to the isotopic dilution technique, they constitute an important tool in clarifying the N cycle. At the Centro de Energia Nuclear na Agricultura (CENA/USP), it was obtained ( 15 NH 4 ) 2 SO 4 enhanced at 3,5% of 15 N atoms, by means of the ionic exchange chromatography technique, which made possible to produce aqua ammonia ( 15 NH 3 aq). Four repetitions were taken to the aqua ammonia production process to use the nitrogen compound in the field experiment. In each process 150g of ammonium sulfate enhanced at 3,5% of 15 N atoms was used, obtaining 31,0 ± 1,6 g of aqua ammonia on the average (80% yield), with the same enhancement. The incidence of isotopic dilution has not been observed during the procedure, what made the use of such methodology possible. After obtaining the aqua ammonia 15 N through this procedure, it was added to the vinasse (an equivalent to 50 m 3 ha -1 ) in doses that corresponded to 70 kg ha -1 of N-NH 3 aq. The mixture was applied to the sugar-cane straw on the soil's surface, aimed to the crop's fertilization. The compound's isotopic composition was analyzed by means of a spectrometer of masses ANCA-SL Europe Scientific, while the total-N volatilized, by the micro-Kjeldahl. Method. In accordance to the low NH 3 (6,4 ± 1,9 kg ha -1 ) volatilization results, it could be concluded that the application of vinasse and aqua ammonia mixture to the straw on the soil's surface was efficient, due to the vinasse's acid character, which allowed the NH 3 , in presence of the ion H + , to stay in the NH 4 + form in solution. (author)

  5. PROCALCITONIN TESTING IN RHEUMATOLOGY

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    D. V. Bukhanova

    2017-01-01

    Full Text Available Currently, differential diagnosis of systemic bacterial infection and active rheumatic process remains a challenging problem in rheumatology. In the review, current data on the role of procalcitonin biomarker in diagnosis and differential diagnosis of rheumatic diseases (RD and infectious pathology are presented. In particular, some authors recommend procalcitonin (PCT test as a marker of bacterial infection in bones and joints at levels above 0.5 ng/ml; at PCT level below 0.3 ng/ml, infection can be ruled out. In patients with microcrystalline arthritis, data on the significance of PCT for differential diagnosis are contradictory. PCT level doesn’t correlate with systemic lupus erythematosus activity and is elevated only during bacterial infection proportionally to its systematicity. In some studies, elevated PCT level was observed in ANCA-associated vasculitis with high activity without bacterial infection. It was shown that in 80 % of adults with Still’s disease, PCT level was higher than the threshold value even without infection. For patients with RD hospitalized in intensive care units, PCT clearance is a more informative predictive characteristic than its level, regardless of the cause of PCT elevation (infection, injury, severe organ damage, etc.; slowdown of its decrease is a factor of poor prognosis and is associated with higher mortality. At the same time, PCT level positively correlates with the SOFA score in presence of bacterial infection. For some rheumatic diseases, the threshold PCT value at which the test has optimal sensitivity and specificity is yet to be established. Nonetheless, PCT should be evaluated in relation to the clinical picture and data of additional examinations. The effect of various therapy methods used in rheumatology on PCT level requires further research.

  6. Human neutrophils in auto-immunity.

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    Thieblemont, Nathalie; Wright, Helen L; Edwards, Steven W; Witko-Sarsat, Véronique

    2016-04-01

    Human neutrophils have great capacity to cause tissue damage in inflammatory diseases via their inappropriate activation to release reactive oxygen species (ROS), proteases and other tissue-damaging molecules. Furthermore, activated neutrophils can release a wide variety of cytokines and chemokines that can regulate almost every element of the immune system. In addition to these important immuno-regulatory processes, activated neutrophils can also release, expose or generate neoepitopes that have the potential to break immune tolerance and result in the generation of autoantibodies, that characterise a number of human auto-immune diseases. For example, in vasculitis, anti-neutrophil cytoplasmic antibodies (ANCA) that are directed against proteinase 3 or myeloperoxidase are neutrophil-derived autoantigens and activated neutrophils are the main effector cells of vascular damage. In other auto-immune diseases, these neutrophil-derived neoepitopes may arise from a number of processes that include release of granule enzymes and ROS, changes in the properties of components of their plasma membrane as a result of activation or apoptosis, and via the release of Neutrophil Extracellular Traps (NETs). NETs are extracellular structures that contain chromatin that is decorated with granule enzymes (including citrullinated proteins) that can act as neo-epitopes to generate auto-immunity. This review therefore describes the processes that can result in neutrophil-mediated auto-immunity, and the role of neutrophils in the molecular pathologies of auto-immune diseases such as vasculitis, rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). We discuss the potential role of NETs in these processes and some of the debate in the literature regarding the role of this phenomenon in microbial killing, cell death and auto-immunity. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Serum biomarkers are similar in Churg-Strauss syndrome and hypereosinophilic syndrome.

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    Khoury, P; Zagallo, P; Talar-Williams, C; Santos, C S; Dinerman, E; Holland, N C; Klion, A D

    2012-09-01

    Churg-Strauss syndrome (CSS) and hypereosinophilic syndrome (HES) overlap considerably in clinical presentation. A reliable means of distinguishing between these groups of patients is needed, especially in the setting of glucocorticoid therapy. A retrospective chart review of 276 adult subjects referred for evaluation of eosinophilia > 1500/μl was performed, and subjects with a documented secondary cause of eosinophilia or a PDGFR -positive myeloproliferative neoplasm were excluded. The remaining subjects were assessed for the presence of American College of Rheumatology (ACR) criteria. Laboratory and clinical parameters were compared between subjects with biopsy-proven vasculitis (CSS; n = 8), ≥4 ACR criteria (probable CSS; n = 21), HES with asthma and/or sinusitis without other CSS-defining criteria (HESwAS; n = 20), HES without asthma or sinusitis (HES; n = 18), and normal controls (n = 8). Serum biomarkers reported to be associated with CSS were measured using standard techniques. There were no differences between the subjects with definite or probable CSS or HES with respect to age, gender, or maintenance steroid dose. Serum CCL17, IL-8, and eotaxin levels were significantly increased in eosinophilic subjects as compared to normal controls, but were similar between the eosinophilic groups. Serum CCL17 correlated with eosinophil count (P < 0.0001, r = 0.73), but not with prednisone dose. In patients with a history of asthma and sinusitis, distinguishing between ANCA-negative CSS and PDGFR-negative HES is difficult because of significant overlap in clinical presentation and biomarker profiles. Published 2012. This article is a U.S. Government work and is in the public domain in the USA.

  8. Present and future management of anti-neutrophil cytoplasmic antibody associated vasculitis: how therapy changed the prognosis

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    Massimo L’Andolina

    2013-03-01

    Full Text Available Anti-neutrophil cytoplasmic antibody associated vasculitis is part of a multi-systemic idiopathic, small vessel pouci-immune vasculitis. Given the heterogeneous spectrum of the disease, and the need to update therapeutic protocols, the aim of this review was to evaluate clinical-diagnostic approaches. We examined statistical data available in the literature, in particular the 2010 review of St. Hamour et al. Management of Anca-associated Vasculitis, published in Therapeutics and Clinical Risk Management. Acute immunosuppressive therapy and long-term maintenance, with the use of prednisolone, have significantly changed the prognosis of this disease, particularly compared with the 1970s before the introductions of steroids and cyclophosphamide. New drugs such as rituximab, monoclonal antibodies and other modulating immune system molecules are entering clinical use, and experience will confirm whether or not therapeutic guidelines are appropriate. The current diagnostic tools, ranging from laboratory and autoimmune tests, chest X-ray, broncho-alveolar lavage to capillaroscopy, allow prompt diagnosis and early treatment through a first phase of induction-remission, and a second phase of maintenance. There are, however, recurrent and refractory forms of the disease that require long-term immunosuppression and further research into this is merited. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, have meant that outcomes have been maintained while toxicity has been reduced.

  9. Patients with IgA nephropathy exhibit high systemic PDGF-DD levels.

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    Boor, Peter; Eitner, Frank; Cohen, Clemens D; Lindenmeyer, Maja T; Mertens, Peter R; Ostendorf, Tammo; Floege, Jürgen

    2009-09-01

    Platelet-derived growth factor (PDGF) is a central mediator of mesangioproliferative glomerulonephritis (GN). In experimental mesangioproliferative GN, PDGF-DD serum levels, unlike PDGF-BB, increased up to 1000-fold. We assessed disease activity in 72 patients with GN, established a novel PDGF-D ELISA and then determined their PDGF-DD levels. In parallel, we studied renal PDGF-DD mRNA expression by RT-PCR. PDGF-DD serum levels in patients with IgA nephropathy (IgAN) were significantly higher (1.67 +/- 0.45 ng/ml) and in patients with lupus nephritis significantly lower (0.66 +/- 0.86 ng/ml) compared to healthy controls (1.17 +/- 0.46 ng/ml), while patients with focal segmental glomerulosclerosis, membranous GN and ANCA-positive vasculitis did not differ from controls. The subgroup of IgAN patients with elevated PDGF-DD levels (27% of samples) did not differ in their clinical features from those with normal PDGF-DD levels. In IgAN patients with repetitive PDGF-DD determinations, most exhibited only minor fluctuations of serum levels over time. Intrarenal PDGF-DD mRNA expression did not differ between controls and patients, suggesting an extrarenal source of the elevated PDGF-DD in IgAN. Serum PDGF-DD levels were specifically elevated in patients with IgAN, in particular in those with early disease, i.e. preserved renal function. Our data support the rationale for anti-PDGF-DD therapy in mesangioproliferative GN.

  10. [IgG4-related disease - a case report].

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    Milczarek-Banach, Justyna; Brodzińska, Kinga; Jankowska, Anna; Ambroziak, Urszula; Szczepankiewicz, Benedykt; Nałęcz-Janik, Jolanta; Miśkiewicz, Piotr

    2017-09-29

    Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years. Graves' orbitopathy and systemic vasculitis were suspected, however both were excluded (negative antibodies results: anty-TSHR, ANCA, ANA). Serologic investigation of Sjögren's syndrome was also negative. In Magnetic Resonance Imaging (MRI) of orbits there were described bilateral mild extension of lateral rectus muscles, normal signal of adipose tissue and bilateral lacrimal glands enlargement. Moreover, increased IgG4 serum levels were detected. The material derived from perinasal sinuses surgery was analyzed in histopathology examination with immunohistochemical staining, which revealed characteristic features of chronic inflammatory process and increased numbers of IgG4 - positive plasma cells (>50 in a large field of view). The diagnosis of IgG4-RD was established. Because of non-effective oral methylprednisolone therapy in the past, the patient was referred to Clinic of Rheumatology for further treatment. After the therapy with methylprednisolone and azathioprine there were observed the significant reduction of symptoms. Because of lack of characteristic symptoms of IgG4- RD, it should be always considered in differential diagnosis of chronic inflammatory diseases of various organs.

  11. Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.

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    Danlos, François-Xavier; Rossi, Giovanni Maria; Blockmans, Daniel; Emmi, Giacomo; Kronbichler, Andreas; Durupt, Stéphane; Maynard, Claire; Luca, Luminita; Garrouste, Cyril; Lioger, Bertrand; Mourot-Cottet, Rachel; Dhote, Robin; Arlet, Jean-Benoit; Hanslik, Thomas; Rouvier, Philippe; Ebbo, Mikael; Puéchal, Xavier; Nochy, Dominique; Carlotti, Agnès; Mouthon, Luc; Guillevin, Loïc; Vaglio, Augusto; Terrier, Benjamin

    2017-10-01

    Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. RECUENTO DE COLIFORMES Y Escherichia coli EN CANALES BOVINAS SOMETIDAS A TRATAMIENTOS FÍSICOS Y QUÍMICOS

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    EDUARDO JAVID CORPAS-IGUARÁN

    Full Text Available Se evaluó el recuento de coliformes totales y Escherichia coli en canales bovinas sometidas a diferentes tratamientos que incluyeron combinaciones de los métodos de lavado, desinfección y vaporización, utilizando el diseño completamente aleatorizado de un factor con 3 repeticiones. El muestreo se realizó mediante frotis de superficie en tres áreas diferentes de la muestra (espalda, pecho y anca, que fueron posteriormente analizadas mediante el método de recuento en placa. Al aplicar secuencialmente los métodos de remoción microbiana se apreció la disminución progresiva en el recuento de coliformes totales y E. coli, y en la variabilidad de los datos, principalmente en los tratamientos C, D y E. La prueba de contrastes múltiples de Friedman mostró la inexistencia de diferencias estadísticas entre los recuentos de los tratamientos C y E en la muestra 2, indicando la ineficacia de la vaporización como coadyuvante de la acidificación para disminuir el recuento de los microorganismos de estudio. Las pruebas de comparación en muestras independientes mostraron resultados idénticos a la prueba de Friedman, excepto para el recuento de E. coli entre los tratamientos D y E, sugiriendo que la vaporización tiene efecto significativo para potenciar la disminución del recuento del microorganismo en las canales.

  13. Competition influence in the segregation of the trophic niche of otariids: a case study using isotopic Bayesian mixing models in Galapagos pinnipeds.

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    Páez-Rosas, Diego; Rodríguez-Pérez, Mónica; Riofrío-Lazo, Marjorie

    2014-12-15

    The feeding success of predators is associated with the competition level for resources, and, thus, sympatric species are exposed to a potential trophic overlap. Isotopic Bayesian mixing models should provide a better understanding of the contribution of preys to the diet of predators and the feeding behavior of a species over time. The carbon and nitrogen isotopic signatures from pup hair samples of 93 Galapagos sea lions and 48 Galapagos fur seals collected between 2003 and 2009 in different regions (east and west) of the archipelago were analyzed. A PDZ Europa ANCA-GSL elemental analyzer interfaced with a PDZ Europa 20-20 continuous flow gas source mass spectrometer was employed. Bayesian models, SIAR and SIBER, were used to estimate the contribution of prey to the diet of predators, the niche breadth, and the trophic overlap level between the populations. Statistical differences in the isotopic values of both predators were observed over the time. The mixing model determined that Galapagos fur seals had a primarily teutophagous diet, whereas the Galapagos sea lions fed exclusively on fish in both regions of the archipelago. The SIBER analysis showed differences in the trophic niche between the two sea lion populations, with the western rookery of the Galapagos sea lion being the population with the largest trophic niche area. A trophic niche partitioning between Galapagos fur seals and Galapagos sea lions in the west of the archipelago is suggested by our results. At intraspecific level, the western population of the Galapagos sea lion (ZwW) showed higher trophic breadth than the eastern population, a strategy adopted by the ZwW to decrease the interspecific competition levels in the western region. Copyright © 2014 John Wiley & Sons, Ltd.

  14. Local esperience on frequency and significance of immunological overlap in the field of primary biliary cirrhosis (PBC

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    Caterina Defendenti

    2008-06-01

    Full Text Available The aim of this study was to investigate the frequency and quality of immunological overlap in serum AMA M2 positives. During the period January 2004 - December 2006, out of 3000 sera evaluated, 40 were AMA M2 positive. Six of these (15% were without immunological overlap; they had clinical, histological and biochemical features of classical PBC with a normal length of life and ursodeoxycholic acid responding. In other 16 patients (40%, there was an association with antibody against antigens as SLA/LP, gp210 and Sp100, that causes the overlap syndrome AIH/PBC with various level of seriousness.The association with anti SLA/LP or with high level of autoantibody anti-Sp100 (index ELISA>10 waspresent in the patients investigated, as AIH type I°. Patients positive for anti gp210 with low anti-Sp100 index had instead a weak overlap AIH/PBC with slow evolving aspects. Nobody was positive for anti-LKM1, anti-LC, PML, or LBR.The remaining 45% of sera were positive for ANA, ENA,ANCA, with different clinical features. The antibody anti-SSARo52 and anti-centromere were frequent (17% and modified the liver disease evolution.The most serious clinical case was a double overlap anti-centromere/SSARo52 with biochemical and histological AIH symptoms. Other ENA patterns did worsen the prognosis of PBC but showed or were associated with systemic diseases, often after a long asymptomatic period.The ANA IFI positive with a speckled pattern ENA negative, in other 7 patients (17% worsened the long term prognosis because they expressed an overlap AIH/PBC with slowly evolving aspects.The most serious and intense clinical picture, with transplant perspective, was the association of the AMA M2 with anti Sp100 (index > 10 and pANNA. Four case were male (10% but females were prevalent.

  15. Autoimmune liver serology: current diagnostic and clinical challenges.

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    Bogdanos, Dimitrios-P; Invernizzi, Pietro; Mackay, Ian-R; Vergani, Diego

    2008-06-07

    Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseases (AiLD), namely autoimmune hepatitis types 1 and 2 (AIH-1 and 2), primary biliary cirrhosis (PBC), and the sclerosing cholangitis variants in adults and children. AIH-1 is specified by anti-nuclear antibody (ANA) and smooth muscle antibody (SMA). AIH-2 is specified by antibody to liver kidney microsomal antigen type-1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1). SMA, ANA and anti-LKM antibodies can be present in de-novo AIH following liver transplantation. PBC is specified by antimitochondrial antibodies (AMA) reacting with enzymes of the 2-oxo-acid dehydrogenase complexes (chiefly pyruvate dehydrogenase complex E2 subunit) and disease-specific ANA mainly reacting with nuclear pore gp210 and nuclear body sp100. Sclerosing cholangitis presents as at least two variants, first the classical primary sclerosing cholangitis (PSC) mostly affecting adult men wherein the only (and non-specific) reactivity is an atypical perinuclear antineutrophil cytoplasmic antibody (p-ANCA), also termed perinuclear anti-neutrophil nuclear antibodies (p-ANNA) and second the childhood disease called autoimmune sclerosing cholangitis (ASC) with serological features resembling those of type 1 AIH. Liver diagnostic serology is a fast-expanding area of investigation as new purified and recombinant autoantigens, and automated technologies such as ELISAs and bead assays, become available to complement (or even compete with) traditional immunofluorescence procedures. We survey for the first time global trends in quality assurance impacting as it does on (1) manufacturers/purveyors of kits and reagents, (2) diagnostic service laboratories that fulfill clinicians' requirements, and (3) the end-user, the physician providing patient care, who must properly interpret test results in the overall clinical context.

  16. Polyarteritis Nodosa: The Angiography usefulness

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    Jorge Pires

    2015-04-01

    Full Text Available A poliarterite nodosa (PAN é uma vasculite de médias e pequenas artérias que se pode manifestar por atingimento de vários órgãos, devendo o diagnóstico ser efetuado com base na clínica, exames laboratoriais e estudo histológico do órgão envolvido. Apresenta-se o caso de uma mulher de 64 anos, caucasiana, com quadro de febre elevada e mialgias nos membros inferiores com cerca de duas semanas de evolução. À observação identificou-se livedo reticularis e hipertensão arterial de difícil controlo. Na avaliação laboratorial destacava-se anemia normocítica e normocrómica e elevação de parâmetros de fase aguda. Efetuada investigação de causa infeciosa e neoplásica que foi inconclusiva. Os anticorpos anti-citoplasma dos neutrófilos (ANCAs foram negativos. As biópsias cutânea e muscular não apoiaram o diagnóstico de vasculite. Realizada arteriografia abdominal que mostrou na vascularização distal de todos os ramos viscerais microaneurismas compatíveis com PAN. A doente foi medicada com prednisolona 1mg/kg/dia, verificando-se franca melhoria clínico-laboratorial. Destaca-se assim a importância da realização da arteriografia nos casos suspeitos de PAN em que este diagnóstico não é possível comprovar através da histologia ou angio TC.

  17. Anti-neutrophil cytoplasmic antibody-associated vasculitis with renal involvement: Analysis of 89 cases.

    Science.gov (United States)

    Caravaca-Fontán, Fernando; Yerovi, Estefanía; Delgado-Yagu E, María; Galeano, Cristina; Pampa-Saico, Saúl; Tenorio, Maria Teresa; Liaño, Fernando

    2017-01-06

    The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with renal involvement are associated with high morbi-mortality. In this study we analyse if the prognosis of these diseases have improved in recent years, and which factors influence the outcomes. Retrospective single-centre observational study, which included all patients diagnosed with microscopic polyangiitis and granulomatosis with polyangiitis with renal involvement in the last 25 years. Demographic, clinical and biochemical parameters of prognostic interest were recorded. The differences between four chronological periods were analysed, along with the determinants of a poor outcome (death or end-stage renal disease). Eighty-nine patients were included (mean age 64±15 years). Sixty-four patients (72%) had microscopic polyangiitis and 25 (28%) granulomatosis with polyangiitis. During the study period, 37 (42%) patients died. Through Cox regression analysis, the best determinants of mortality were the initial glomerular filtration rate (HR 0.911; P=.003), Charlson comorbidity index (HR 1.513; P<.0001) and tobacco smoking (HR 1.816; P=.003). 35% developed end-stage renal disease, and the best determinants (by competing-risk regression) were: initial glomerular filtration rate (sub-hazard ratio [SHR]: 0.791; P<.0001), proteinuria (SHR: 1.313; P<.0001), and smoking status (SHR: 1.848; P=.023). No differences were found in patients' mortality or renal survival between the different study periods. Prognosis of anti-neutrophil cytoplasm antibodies vasculitis with renal involvement treated with conventional immunosuppressive therapy remains unsatisfactory, and continues to have increased long-term complications and mortality. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  18. Stress and Disease Onset in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

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    Christina V. Golemati

    2017-12-01

    Full Text Available ObjectiveTo explore the potential contribution of stress as a trigger for disease onset in patients with antineutrophil cytoplasmic antibody (ANCA associated vasculitis (AAV.Methods53 AAV and 85 rheumatoid arthritis (RA patients as well as 53 healthy controls (HC were thoroughly asked for the number and impact of stressful life events, coping strategies, and available social support 12 months prior to disease onset. Anxiety, depression, personality dimensions, insomnia, and fatigue were also determined.ResultsAAV patients reported higher scoring of the impact of stressful life events compared to the RA and HC group prior to disease onset (2.8 ± 3.1 vs 1.8 ± 2.1 vs 1.7 ± 2.3, p-values: 0.047 and 0.053, respectively. While the number of reported stressful events was found to be significantly higher in AAV vs RA patients but not HC, certain coping strategies and social support features were more commonly implemented by AAV patients compared to HC, but not RA patients. As far as personality and other psychosocial characteristics, AAV patients displayed significantly higher psychoticism traits compared to RA, with no other differences being detected between AAV patients and both RA and HC. After adjusting for potential cofounders, scoring of the impact of stressful life events >3 was independently associated with AAV development compared to both RA and HC [ORs (95% CI: 4.6 (1.6–13.4 and 4.4 (1.0–19.0, respectively].ConclusionThe perceived impact of stressful life events prior to disease onset emerged as a contributing factor for AAV development.

  19. Superficial basal cell carcinoma treated with 70% trichloroacetic acid applied topically: a case study

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    Chiriac A

    2017-02-01

    Full Text Available Anca Chiriac,1–3 Piotr Brzezinski,4 Cosmin Moldovan,5 Cristian Podoleanu,6 Marius Florin Coros,7 Simona Stolnicu8 1Department of Dermatology, Nicolina Medical Center, Iasi, 2Department of Dermatology, Apollonia University, Iasi, 3Department of Dermatology, P. Poni Research Institute, Romanian Academy Iasi, Romania; 4Department of Dermatology, 6th Military Support Unit, Utska, Poland; 5Department of Histology, 6Department of Cardiology, 7Department of Surgery, 8Department of Pathology, University of Medicine and Pharmacy of Targu‑Mures, Targu-Mures, Romania Background: Basal cell carcinoma (BCC is the most common form of skin cancer, affecting millions of people worldwide. The treatment concept for BCCs is the surgical one, but it is costly, as such, searching for alternative medical therapeutics is justified. Aim: To highlight the efficacy of high concentration (70% trichloroacetic acid (TCA as a choice therapy for low-risk BCC. Method and patient: Authors present, for the first time, the use of a high concentration TCA applied once a week for 2 consecutive weeks with a toothpick, on a patient with BCC on the right preauricular area. Results: On examination 4 weeks later, the lesion was not clinically and dermatoscopically evidenced. Conclusion: High concentration TCA could be an effective and safe, non-invasive choice of therapy for low-risk BCC, easy to perform, not expensive, with good cosmetic results, especially for patients who are not likely to undergo invasive or expensive treatments. Keywords: basal cell carcinoma, therapy, trichloroacetic acid, dermatology

  20. Evaluating the utility of serological testing in laryngotracheal stenosis.

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    Hall, S Ryan; Allen, Clint T; Merati, Albert L; Mayerhoff, Ross M

    2017-06-01

    Whereas mechanical (traumatic) causes of laryngotracheal stenosis (LTS) are identified based on history, autoimmune laryngotracheal stenosis (aLTS) and idiopathic laryngotracheal stenosis (iLTS) are often more difficult to differentiate. The objective of this study was to evaluate serologic testing in a large cohort of nonmechanical LTS patients to determine which tests, if any, lead clinicians to the etiology of the LTS. Retrospective chart review. This study reviewed nonmechanical LTS patients seen at a tertiary medical center from 2007 to 2014. Data were obtained on patient demographics, associated preexisting autoimmune conditions, comorbidities, intubation history, and serologic testing. Ninety-two records were reviewed. Twenty-three (25%) patients were found to have autoimmune disease; 69 (75%) met criteria for iLTS. A history of cigarette smoking was more significant in the aLTS group than the iLTS group (P testing was equivocal between the two cohorts. Differentiating iLTS from aLTS has proven difficult. The lack of information about the two entities has resulted in variability in the diagnostic workup to distinguish them. This study's finding of a more significant smoking history in the aLTS group correlates with the literature, which suggests an inflammatory effect of smoking cigarettes and an association with autoimmune disease. The only significant cohort of patients in this study found to have positive serological testing correlated with a diagnosable condition responsible for LTS was GPA patients with positive ANCA. 4. Laryngoscope, 127:1408-1412, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  1. Oclusão da artéria central da retina em paciente com poliangeíte microscópica

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    Cláudia Gallicchio Domingues

    2015-12-01

    Full Text Available RESUMO A poliangeíte microscópica é uma vasculite necrotizante sistêmica que acomete arteríolas, capilares e vênulas, mas também pode atingir pequenas e médias artérias. É considerada uma doença rara, idiopática e autoimune. Diversas anormalidades oculares e sistêmicas estão associadas às oclusões arteriais retinianas. Dentre as doenças vasculares do colágeno, a literatura cita como possíveis causas de obstrução das artérias retinianas o lúpus eritematoso sistêmico, a poliarterite nodosa, a arterite de células gigantes, a granulomatose de Wegener e a granulomatose linfóide de Liebow. Até o presente momento, não se encontrou na literatura relatos da associação de casos de oclusão arterial retinana associados à PAM. Os autores relatam o caso de um paciente com poliangeíte microscópica que apresentou comprometimento renal importante e oclusão da artéria central da retina unilateral. Atenta-se para a inclusão de pesquisa da PAM, através do p-ANCA, na avaliação de possível origem sistêmica em pacientes acometidos por oclusão arterial retiniana.

  2. The Serbian state in the work of Byzantine historian Doucas

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    Nikolić Maja

    2007-01-01

    Full Text Available While the first two chapters of Doucas's historical work present a meagre outline of world history - a sketch which becomes a little more detailed from 1261 on, when the narration reaches the history of the Turks and their conquests in Asia Minor - the third chapter deals with the well-known battle of Kosovo, which took place in 1389. From that point on, the Byzantine historian gives much important information on Serbia, as well as on the Ottoman advances in the Balkans, and thus embarks upon his central theme - the rise of the Turks and the decline of Byzantium. Doucas considers the battle of Kosovo a key event in the subjugation of the Balkan peoples by the Turks, and he shows that after the battle of Kosovo the Serbs were the first to suffer that fate. At the beginning, Doucas says that after the death of Orhan, the ruler (o archgos of the Turks, his son and successor Murad conquered the Thracian towns, Adrianople and the whole Thessaly, so that he mastered almost all the lands of the Byzantines, and finally reached the Triballi (Triballous. He devastated many of their towns and villages sending the enslaved population beyond Chersonesus, until Lazar, son of King Stefan of Serbia (Serbias, who ruled (kraleyōn in Serbia at that time decided to oppose him with all the might he could muster. The Serbs were often called Triballi by Byzantine authors. For the fourteenth century writers Pachymeres, Gregoras, Metochites and Kantakouzenos the Serbs were Triballi. However, Pachymeres and Gregoras refer to the rulers of the Triballi as the rulers of Serbia. Fifteenth century writers, primarily Chalcondyles and Critobulos, use only that name. It seems, nevertheless, that Doucas makes a distinction between the Triballi and the Serbs. As it is known, the conquest of the Serbian lands by the Turks began after the battle on the river Marica in 1371. By 1387. the Turks had mastered Serres(1388 Bitola and Štip (1385, Sofia (1385, Niš (1386 and several

  3. Selected Abstracts of the 6th International Congress of UENPS; Valencia (Spain; November 23rd-25th 2016; Session “Outcomes of prematurity”

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    --- Various Authors

    2016-11-01

    Full Text Available Selected Abstracts of the 6th International Congress of UENPS; Valencia (Spain; November 23rd-25th 2016; Session “Outcomes of prematurity”ABS 1. CHILDHOOD NEURODEVELOPMENTAL OUTCOME IN LOW BIRTH WEIGHT INFANTS WITH POSTLIGATION CARDIAC SYNDROME AFTER DUCTUS ARTERIOSUS CLOSURE: 5-YEAR FOLLOW-UP • M.C. Bravo, M. Ybarra, R. Madero, A. PellicerABS 2. PARENT CARE TAGS: ENGAGING PARENTS IN CARE ON THE NEONATAL UNIT • R. Homer, L. RattenburyABS 3. STRESS BIOMARKERS AND PSYCHOSOCIAL FACTORS AS PREMATURITY PREDICTORS IN THREATENED PRETERM LABOR • A.C. García-Blanco, M. Vento, P. Sáenz, V. Diago, C. Cháfer-PericásABS 4. PREDICTION OF WEEK OF CHILDBIRTH FOR WOMEN WITH THREATENED PRETERM LABOR • C. Cháfer-Pericás, M. Vento1, V. Diago, V. Serrano-De la Cruz, D. Hervás, A. García-BlancoABS 5. DELIVERY ROOM CARDIOPULMONARY RESUSCITATION (CPR AT THE LIMIT OF VIABILITY • S. Zeballos Sarrato, S. Villar, I. Pescador, M. Carrón, A. Rodriguez, M. Sánchez LunaABS 6. QUALITY OF LIFE IN PREMATURE BABIES FROM MOTHER’S PERSPECTIVE • A. Bivoleanu, A. Avasiloaiei, M. StamatinABS 7. PERINATAL MANAGEMENT AND MORTALITY RATES AT THE LIMIT OF VIABILITY • S. Zeballos Sarrato, E. Sanz, I. Pescador, S. Villar, N. Navarro, M. Sánchez LunaABS 8. OUTCOMES OF LATE PRETERM NEWBORNS • D. Stoniene, J. Ribeliene, S. Malik, J. Tomkeviciute, R. TamelieneABS 9. MAGNESIUM SULPHATE, ANTENATAL STEROIDS, CORD CLAMPING, THERMOREGULATION (MASCOT • A. Ansary, D. AnandABS 10. FOLLOW-UP AT AGE 8 YEARS OF A POST-DISCHARGE NUTRITION RCT IN PRETERM INFANTS • C.A. Ruys, H.N. LafeberABS 11. NEONATAL OUTCOMES OF PRETERM BABIES (23-32 WEEKS GESTATION CLASSIFIED BY THE UNDERLYING AETIOLOGIES: A RETROSPECTIVE STUDY IN A SPECIALISED NEONATAL UNIT • F. Jaffar, S. Sankaran, S. Tan, A. ShennanABS 12. ARE THERE PREDICTIVE PARAMETERS FOR THE OCCURRENCE OF RETINOPATHY OF PREMATURITY – ROP? • V. Filip, C. LazarABS 13. POSTNATAL GROWTH OF PRETERM CHILDREN

  4. Theoretical points of view on the notion of cause (consideration of contracts in the Serbian doctrine of civil law till the adoption of the law on obligations

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    Dudaš Atila

    2011-01-01

    contract in the Serbian literature. In contrast to the period before the adoption of the Law on Obligations, after its adoption a tendency of abandonment of points of view disavowing any legal relevance of cause of contract can be recognized and the majority of contemporary authorities argue that the cause of contract is a necessary legal institution, although they may differ in the conceptual determination of this notion. In the period after the adoption of the Law on Obligations in the Serbian literature of law of contract many authorities analyzed various aspects of cause of contract, especially Professors Stojan Cigoj, Ljubiša Milošević, Živomir Đorđević, Boris Vizner, Martin Vedriš, Želimir Šmalcelj, Jakov Radišić, Slobodan Perović, Jožef Salma, Oliver Antić and others. The author in this paper gives an overview of the points of view of authorities in the Serbian literature of law contract on the notion of cause of contract who marked the period until the adoption of the Law on Obligations, namely the works of Professors Dragoljub Aranđelović, Živojin Perić, Lazar Marković, Andrija Gams, Stevan Jakšić, Vladimir Kapor and Mihajlo Konstantinović.

  5. Church of St. Peter and Paul in the village under Kopaonik

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    Raičević Slobodan

    2009-01-01

    Full Text Available In the border of the municipality towards Kosovo, Kuršumlija preserved the St. Cemetery's church of Peter and Paul in village Lukovo near the famous rehabilitation center Lukovo's spa. Church is one nave with a semicircular apse at the east vaults with semicircular arch reinforced with cross sec­tion curve, which relies on a few strong pilasters placed inside long walls. According to folk tradition the church was built in the middle ages and served to diggers and foundries of lead ores of King Milutin. Upon termination of exploitation ore the church neglected and in the 19th century it suffered its first renewal 1871st and another 1895th year. Saved epigraphic inscription with carved year of renewal (1895th, icons and wall decor of the church provide information about reliably judging its recent history and artistic value. Soon after the bricklaying work 1895th Church has received from the altar wall of icons, and after that, and mural painting small artistic value. The crown of the vault is painted Pantocrator, about whose performances are painted scenes of the Transfiguration, Ascension of Christ, Vavedenje and Entry into Jerusalem. On the western wall are shown the Dormition of the Mother of God and in the first zone are shown standing figures of Sts. Constantine and Helena, St. Simon Mir refills and St. Prince Lazar - kefaloforos. On the triumphal arch are Old. Testament scenes of hospitality and sacrifice of Abraham and in the apse bust of God Savaot. In the zone of standing figures are shown Sts. Druid, St. Czar Uroš and St. King Stephen, St. Sunday, St. Petka, St. Procopius, and St. George. From the figures standing at the altar of bishops are shown St. John Chrysostom, St. Sava Serbian, St. Basil the Great and St. Athanasius. There was saved the altar compartments with icons. At the czar's gate are shown Blagovesti and in the order of main icons: Christ Almighty, Mother of God with Christ, St. John the Forerunner, icons of patron St

  6. The painting of the Veluća's church

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    Andrejić Živojin R.

    2013-01-01

    beginning of the 13th century by the archiepiscope Sava I. that is why the Phase concept of in the narthex of the family memory is connected to the death of the youngest and early deceased Oliver, the grandson of the founder and the descendant of this lineage of rulers. The way of virtue was allusively presented, that he was treading accompanied by saints in order to reach Heaven. The point of Oliver's death had been ideally, symbolically and artistically well presented through phases by the choice of the saints who clearly mark the calendar of the destiny of death and burial of young Oliver: St Archangel Gabriel, Jully 13 th, St Kilik and Julita, Jully 15th, St Marina, July 17th. The painting of the Veluća's narthex, for the first time in the Serbian narrative art of its age has a painting program where the deceased, young gentleman Oliver, then his uncle Jovan Dragaš and Father Konstantin, allusively presented through phases connected with saints who represented the dates declaration, July 13th, their death July 15th and funeral, July 17th where over their graves their portrait would be painted, that associates on incurable grief of the whole family. The exquisite narrative of the frescoes in Veluća has a direct analogy in the monuments of Serbia in the first half of 14th century and the age of the Empire and thus it threads forcefully together with the chronology of its founders. In accordance with that, it can naturally be stated that the frescoes of V eluća stand out from the Moravski style by style and iconography and joins the narrative style, and the architectural features mark this church as a predecessor of building style in the age of Prince Lazar.

  7. Patriarch Ephrem: A late medieval saintly cult

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    Popović Danica

    2006-01-01

    extremely complex, even dramatic, political and social circumstances created by the conflict between the patriarchates of Serbia and Constantinople, on the one hand, and rivalries between local lords, on the other. There is a difference of interpretation as to his role as the holder of patriarchal office. The latest findings appear to suggest that Ephrem, as an exponent of Mount Athos, loyal to the Patriarchate of Constantinople and close to Vuk Brankovic, was unacceptable to the Lazarević dynasty who emerged victorious in the power straggles in Serbia. Their victory was crowned with the creation of the cult of the holy prince Lazar, a Kosovo martyr. Although a supporter of the defeated side, patriarch Ephrem, as an unquestionable spiritual authority and very deserving personage, was included among the saints shortly after his death. His cult, however, had never been made complete. He was given a Life and service, but the attempted elevation of his body, i.e. creation of the cult of his relics, was thwarted. The reasons, political in nature, were given in the form of a coded hagiographical message in his Life composed by bishop Mark, an active protagonist in all the events. .

  8. Despot Stefan and Byzantium

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    Kalić Jovanka

    2006-01-01

    Full Text Available The topic of this paper is one aspect of the relationship between Serbia and Byzantium at the beginning of the 15th Century, during the so-called "despot period" of the reign of Stefan Lazarević (1402-1427, namely the fate of the Byzantine title of Despots' in Serbia against the background of the political situation in the Balkans at the time of Turkish domination. Knez Stefan (1377-1427, Knez Lazar's son, received the title of Despotes according to the procedure long ago established at the Byzantine Court. In Byzantium, this title, which was second in rank only to the title of the Emperor, used to be endowed to the relatives of the imperial dynasty, it was not hereditary and did not depend on the territory ruled by the bearer of the title. It was a personal court title of the highest rank in Byzantium. This honor was bestowed upon the young Knez Stefan in summer of 1402 after his return from the battlefield of Angora (Ankara, where Sultan Beyazid I suffered a disastrous defeat from the hands of the Tatars. The Serbian Knez was solemnly received in Constantinople, a marriage between himself and a sister of the Byzantine Empress was arranged and John VII Palaeologus, the co-regent of the then-absent Emperor Manuel II Palaeologus, endowed him with the title of Despotes. Knez Stefan carried this title till the end of his life. It was held in great honors in Serbia and was broadened in meaning to designate a ruler's title in general, remaining alive among the Serbs even after the fall of the Byzantine Empire. Stefan Lazarević received the dignity of a Despotes once more, in 1410 in Constantinople. All this notwithstanding, the political situation in the South-East of Europe at the beginning of the 15th Century was all but favorable. Some Christian states were conquered by the Turks (Bulgaria, some were vassals of the Sultan (Byzantium, Serbia. Everything depended on the Ottomans. At the time of dynastic conflicts in the Turkish Empire (1403

  9. Theories of cause (consideration of contract in the Serbian doctrine of civil law after the adoption of the Law on Obligations

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    Dudaš Atila

    2012-01-01

    Full Text Available The adoption of the Law on Obligations in 1978 can certainly be considered as an event of watershed importance in respect to the analysis of various theoretical points of view on the notion of cause (consideration of contract in the Serbian literature. The fact that it had not been explicitly regulated in Serbian positive law until the adoption of Law had a profound impact on the evolution of doctrine in this period. The range of differing theoretical points of view was very wide, covering essentially all the relevant streams in the doctrine of cause, from the ones accepting it (so-called causalistic theories, through the ones explaining it solely by the notion of exchange of assets (so-called economic theories, to the ones denying its legal significance in any sense (so called anticausalistic theories. The most distinguished authorities in Serbian literature of contract law, who studied the notion of cause of contract in the period until the promulgation of the Law, were Dragoljub Aranđelović, Živojin Perić, Lazar Marković, Andrija Gams, Stevan Jakšić, Vladimir Kapor and Mihailo Konstantinović. The Law on Obligations explicitly regulates the notion of cause of contract as one of the preconditions of validity and effect of a contract, which had a substantial impact on the evolution of doctrine. On the one hand, being an institution of positive law, there is hardly an author who had not devoted some attention to the notion of cause of contract. In this paper the author analyzes the points of view of Stojan Cigoj, Ljubiša Milošević, Živomir Đorđević, Boris Vizner, Martin Vedriš, Želimir Šmalcelj, Jakov Radišić, Slobodan Perović, Jožef Salma and Oliver Antić. On the other hand, the scope of various, often conflicting, theories on cause of contract in Serbian literature shrank. One can recognize a tendency of abandonment of points of view disavowing any legal relevance of cause of contract and the majority of contemporary

  10. TECHIRGHIOL - 115 YEARS OF BALNEOTHERAPY

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    SCIENTIFIC PROGRAM

    2015-06-01

    Autori: L.E. Stanciu, M. Minea, O. Surdu, V. Marin 1540 - 1600 Dr. Ionescu Valentina Elena, medic primar Recuperare, Medicină Fizică şi Balneologie doctor în Medicină, S.B.R.Techirghiol “Evaluarea efectelor nămolului sapropelic de Techirghiol asupra adipokinelor în osteoartrită” Autori: E.V.Ionescu, L. E. Stanciu, M. Minea, T.V. Surdu 1630 – 1650 Dr. Viorica Marin, medic primar Recuperare, Medicină Fizică şi Balneologie, doctor în Ştiinţe, S.B.R. Techirghiol „Efecte fiziologice şi terapeutice produse de componenta biochimică a aplicaţiei nămolului sapropelic de Techirghiol” Autori: V. Marin, L.E. Stanciu, M. Minea, O. Surdu, T.V. Surdu 1650 – 1710 Dr. Profir Daniela, medic primar Recuperare, Medicină Fizică şi Balneologie, doctor în Ştiinţe, S.B.R.Techirghiol „Efecte fiziologice şi terapeutice produse de componenta biofizică a aplicaţiei nămolului sapropelic de Techirghiol” Autori: D. Profir, L.E. Stanciu, M. Minea, O. Surdu 1710 – 1730 Conf. Univ. Dr. Olga Surdu, medic primar Recuperare, Medicină Fizică şi Balneologie, doctor în Medicină, Universitatea Ovidius Constanţa, Facultatea de Medicină, S.B.R.Techirghiol “Critical review on scientific visibility of studies realised in Balneal and Rehabilitation Sanatorium of Techirghiol” Autori: O. Surdu, M. Surdu, L.E. Stanciu, M. Minea Sunday, 29.06.2014 1000 – 1300 Session IV „Afecţiuni reumatologice – actualităţi şi perspective de tratament balnear” 1000 – 1020Drd. Minea Mihaela medic specialist reumatolog, medic specialist Recuperare, Medicină Fizică şi Balneologie, S.B.R.Techirghiol „Registrul de evidenta a pacienţilor cu boli autoimmune actualităţi şi perspective” Autori: M. Minea, D. Oprea 1020 – 1040 Dr. Oprea Doiniţa medic specialist reumatolog, doctor în Medicină, S.B.R.Techirghiol “Prezentări de cazuri clinice – pacienţi cu afecţiuni reumatologice trataţi în SBRT” Autori: D. Oprea, M. Minea 1040 – 1100 Dr. Viorica

  11. Associations between interleukin-1 polymorphisms and susceptibility to vasculitis: a meta-analysis.

    Science.gov (United States)

    Song, G G; Kim, J-H; Lee, Y H

    2016-05-01

    The objective of this study was to determine whether interleukin-1 (IL-1) polymorphisms are associated with susceptibility to vasculitis. A meta-analysis was conducted to investigate possible associations between IL-1A, IL-1B, and IL-1 receptor antagonist (IL1RN) polymorphisms and vasculitis. A total of 17 studies involving 1384 vasculitis cases [Behçet's disease (BD), IgA vasculitis, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), Kawasaki disease (KD), giant cell arteritis, and Takayasu's arteritis] and 2710 controls were included in the meta-analysis. This analysis showed an association between BD and the TT + TC genotypes of the IL-1A-889 C/T polymorphism in the entire study population [odds ratio (OR) = 0.623, 95 % CI = 0.395-0.981, p = 0.045), and a trend toward an association in a Turkish population (OR = 0.578, 95 % CI = 0.331-1.010, p = 0.054). A meta-analysis of the IL1RN polymorphism revealed no association with vasculitis in all study subjects (OR for IL1RN*2 = 0.904, 95 % CI = 0.626-1.304, p = 0.588). However, stratification by ethnicity revealed a significant association between the IL1RN*2 allele and vasculitis including AAV, BD, KD in Asians (OR = 2.393, 95 % CI = 1.429-4.006, p = 0.001), but not in Caucasian and Turkish populations (OR = 0.776, 95 % CI = 0.487-1.238, p = 0.288; OR = 0.914, 95 % CI = 0.667-1.252, p = 0.576, respectively). No association was found between vasculitis and the IL-1B-511 C/T polymorphism, or the IL-1B+3953 C/T polymorphism. This meta-analysis suggests that the IL-1A-889 C/T polymorphism is associated with susceptibility to BD, and that the IL1RN*2 allele is associated with susceptibility to vasculitis including AAV, BD, and KD in Asians.

  12. [Pharmacotherapy of hyperthyreosis--adverse drug reactions].

    Science.gov (United States)

    Perger, Ludwig; Bürgi, Ulrich; Fattinger, Karin

    2011-06-01

    The antithyroid drugs mainly include thioimidazole (carbimazole, methimazole=thiamazole) and propylthiouracil. After absorption, carbimazole is rapidly metabolized to methimazole and thus switching between these two drugs should not be considered in case of side effects. Furthermore, in case of side effects, sometimes even cross reactions between thioimidazoles and propylthiouracil occur. Common and typical adverse reactions of antithyroid drugs include dose dependent hypothyroidism and thus thyroid function should be repeatedly checked while the patient is on antithyroid drugs. Furthermore, pruritus and rash may develop. In this case, one might try to switch from thioimidazoles to propylthiouracil or vice versa. Antithyroid drugs may cause mild dose dependent neutropenia or severe allergy-mediated agranulocytosis, which typically occurs during the first three months of treatment, has an incidence of 3 per 10,000 patients and cross reactivity between thioimidazoles to propylthiouracil may occur. Rarely, antithyroid drugs can cause aplastic anemia. Mainly propylthiouracil, but sometimes also methimazole may lead to an asymptomatic transient increase in liver enzymes or to severe, even lethal liver injury of cholestatic or hepatocellular pattern. Since propylthiouracil associated liver injury was observed increasingly among children and adolescent, it has been suggested to prefer thioimidazoles for these patients. Because of these potential serious adverse effects, physicians should advise patients to immediately seek medical help if they get a fever or sore throat or malaise, abdominal complaints or jaundice, respectively. Furthermore, arthralgias may develop in 1-5% of patients under both antithyroid drugs. Since arthralgias may be the first symptom of more serious immunologic side effects, it is recommended to stop the antithyroid drug in this case. Drug induced polyarthritis mainly develops during the first month of therapy, whereas ANCA-positive vasculitis is

  13. Sistema de fabrico rápido de implantes ortopédicos Rapid manufacturing system of orthopedics implants

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    Carlos Relvas

    2009-06-01

    Full Text Available Este estudo teve como objectivo o desenvolvimento uma metodologia de fabrico rápido de implantes ortopédicos, em simultaneidade com a intervenção cirúrgica, considerando duas potenciais aplicações na área ortopédica: o fabrico de implantes anatomicamente adaptados e o fabrico de implantes para substituição de perdas ósseas. A inovação do trabalho desenvolvido consiste na obtenção in situ da geometria do implante, através da impressão directa de um material elastomérico (polivinilsiloxano que permite obter com grande exactidão a geometria pretendida. Após digitalização do modelo obtido em material elastomérico, o implante final é fabricado por maquinagem recorrendo a um sistema de CAD/CAM dedicado. O implante após esterilização, pode ser colocado no paciente. O conceito foi desenvolvido com recurso a tecnologias disponíveis comercialmente e de baixo custo. O mesmo foi testado sob a forma de uma artroplastia da anca realizada in vivo numa ovelha. O acréscimo de tempo de cirurgia foi de 80 minutos sendo 40 directamente resultantes do processo de fabrico do implante. O sistema desenvolvido revelou-se eficiente no alcance dos objectivos propostos, possibilitando o fabrico de um implante durante um período de tempo perfeitamente compatível com o tempo de cirurgia.This study, aimed the development of a methodology for rapid manufacture of orthopedic implants simultaneously with the surgical intervention, considering two potential applications in the fields of orthopedics: the manufacture of anatomically adapted implants and implants for bone loss replacement. This work innovation consists on the capitation of the in situ geometry of the implant by direct capture of the shape using an elastomeric material (polyvinylsiloxane which allows fine detail and great accuracy of the geometry. After scanning the elastomeric specimen, the implant is obtained by machining using a CNC milling machine programmed with a dedicated CAD

  14. Caracterización bovinométrica de hembras cebú y cruces con blanco orejinegro, romosinuano y angus.

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    Juan Fernando Medina.

    2005-05-01

    Full Text Available Con el objetivo de caracterizar y determinar algunos factores que podrían ser de gran utilidad a la hora detomar decisiones en una empresa ganadera, se consideraron mediciones cuantitativas como la alzada yamplitud de isquiones, y cualitativas como la condición corporal, ángulo de anca, aplomos, ubre y pezones.Se realizaron análisis de varianza para las variables en cada estado fisiológico (hembras de levante, vacasparidas, vacas paridas preñadas, vacas horras y vacas horras preñadas y se consideraron los efectos de laedad del animal (regresión lineal y cuadrática y grupo genético del animal. Las hembras jóvenes (9 a 12meses tuvieron un peso promedio de 172.0 Kg., y las vacas adultas un promedio de peso entre 415 a 445Kg. En el peso, el efecto de grupo genético, fue altamente significativo en las vacas horras, donde losgrupos genéticos con mayor peso fueron el Cebú y cruce BON x Cebú. Para los otros estados fisiológicosno se encontraron diferencias estadísticas. Para las hembras de levante, las BxC/CxB (n= 100 presentaronmenor condición corporal frente a los CCO/BRH (n= 28, RxC/CxR (n= 50 y 3/4Cx1/4A (n= 15. En lasvacas horras el grupo genético CCO/BRH (n= 328 fue el que presentó la mejor condición corporal. Lasvacas horras preñadas mostraron diferencias entre los grupos genéticos CCO/BRH (n= 273 y AxC/CxA(n= 332. En las vacas paridas, el grupo genético 3/4Cx1/4A (n= 91 obtuvo un promedio superior a losotros grupos analizados. El grupo genético 3/4Cx1/4A presenta los mejores registros en cuanto a cualidadesfísicas en las vacas adultas, lo que confirma su potencial productivo para las zonas groecológicas similaresa las del presente estudio.

  15. Association of Anti-glycan Antibodies and Inflammatory Bowel Disease Course.

    Science.gov (United States)

    Paul, S; Boschetti, G; Rinaudo-Gaujous, M; Moreau, A; Del Tedesco, E; Bonneau, J; Presles, E; Mounsef, F; Clavel, L; Genin, C; Flourié, B; Phelip, J-M; Nancey, S; Roblin, X

    2015-06-01

    The usefulness of anti-glycan antibodies alone or combined with anti-Saccharomyces cerevisiae [ASCA] or perinuclear antineutrophil cytoplasmic [pANCA] antibodies for diagnosis of inflammatory bowel disease [IBD], differentiation between Crohn's disease [CD] and ulcerative colitis [UC], disease stratification including IBD phenotype, and also for determination of the course of the disease, remain unclear. A large panel of serological anti-glycan carbohydrate antibodies, including anti-mannobioside IgG antibodies [AMCA], anti-chitobioside IgA [ACCA], anti-laminaribioside IgG antibodies [ALCA], anti-laminarin [anti-L] and anti-chitine [anti-C] were measured in the serum from a cohort of 195 patients with IBD] [107 CD and 88 UC]. The respective accuracy of isolated or combined markers for diagnosis, disease differentiation, stratification disease phenotype, and severity of the disease course, defined by a wide panel of criteria obtained from the past medical history, was assessed. The positivity of at least one anti-glycan antibody was detected in a significant higher proportion of CD and UC compared with healthy controls [p ACCA [> 51U/ml] and anti-laminarin [> 31U/ml] were significantly linked with a higher association with steroid dependency (odds ratio [OR] =2.0 [1.0-4.0], p = 0.03 and OR = 2.4 [1.1-5.2], p = 0.02, respectively]. We further defined the respective performance of anti-glycan antibodies to discriminate between patients with severe or not severe CD and UC course and determined the associated optimal cut-off values: severe CD course was significantly more likely in case of AMCA > 77U/ml [OR = 4.3; p = 0.002], ASCA > 63U/ml [OR = 3.5; p ACCA > 50U/ml [OR = 2.8; p 52U/ml [OR = 3.4; p = 0.04] and ACCA > 25U/ml [OR = 3.0; p < 0.04]. Anti-glycan antibodies are valuable serological markers, especially AMCA antibodies that may help clinicians to promptly classify patients into high risk for severe disease. Copyright © 2015 European Crohn’s and Colitis

  16. Spotlight on rituximab in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis: current perspectives

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    Moog P

    2015-11-01

    Vasculitis Activity Score was 0. Maintenance therapy with rituximab 375 mg/m2 every 6 months was started. At the last visit after 8 months, the patient was still in remission, with only minor persistent dysesthesia of the left foot and a persistent serum creatinine of 133 µmol/L. Keywords: ANCA, GPA, granulomatosis with polyangiitis, MPA, microscopic polyangiitis, management

  17. [VASCULITIDES IN CHILDHOOD: A RETROSPECTIVE STUDY IN A PERIOD FROM 2002 TO 2012 AT THE DEPARTMENT OF PAEDIATRICS, UNIVERSITY HOSPITAL CENTRE ZAGREB].

    Science.gov (United States)

    Jelusić, Marija; Kostić, Lucija; Frković, Marijan; Davidović, Masa; Malcić, Ivan

    2015-01-01

    The aim of our study was to analyze clinical features, laboratory findings, treatment, course and outcome of different types of vasculitis in children. All children aged up to 18 years that have been diagnosed with a vasculitis disorder from 2002. to 2012. at the Department of Paediatric, University Hospital Centre Zagreb according to EULAR/PRES/PRINTO criteria were included in the study. Vasculitis was diagnosed in 180 children, 101 girls and 79 boys, mean age 7.19 ± 3.7 years, with an average follow-up of 5.58 ± 3.28 years. Most of the children (155 or 86%) were diagnosed with Henoch-Shönlein purpura (HSP), polyarteritis nodosa (PAN) was diagnosed in 6 children (3.3%), isolated cutaneous leukocytoclastic vasculitis in 5 (2.8%), Takayasu arteritis (TA) and Kawasaki disease in 2 (1.1%) respectively, hypocomplementemic urticarial vasculitis in one patient (0.5%) and other types of vasculitis in 10 (5.5%) patients (vasculitides in systemic connective tissue disorders in 7 and unclassified vasculitides in 3 patients). All patients had elevated inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Anti-neutrophil cytoplasmatic antibodies (ANCA) were positive only in one patient, suffering from microscopic polyangiitis. Treatment modality in most patients were NSAIDs, while children with kidney or gastrointestinal system affection were treated with glucocorticoids and/or immunosuppresive drugs. Biological therapy (anti-CD20, rituximab) was used in patients with most severe symptoms. One child (0.56%), suffering from microscopic polyangiitis, died due to kidney failure during the follow-up. Forty patients (22.6%) had one disease relapse, while 6 (3.4%) had two relapses. In conclusion, we found some differences in laboratory parameters (e.g. lower incidence of elevated antistreptolysin O titer in HSP) and epidemiological data (e.g. higher prevalence of PAN in female children) in comparison to data from available studies, while other clinical

  18. Contrasting association of a non-synonymous leptin receptor gene polymorphism with Wegener's granulomatosis and Churg-Strauss syndrome.

    Science.gov (United States)

    Wieczorek, Stefan; Holle, Julia U; Bremer, Jan P; Wibisono, David; Moosig, Frank; Fricke, Harald; Assmann, Gunter; Harper, Lorraine; Arning, Larissa; Gross, Wolfgang L; Epplen, Joerg T

    2010-05-01

    There is evidence that the leptin/ghrelin system is involved in T-cell regulation and plays a role in (auto)immune disorders such as SLE, RA and ANCA-associated vasculitides (AAVs). Here, we evaluate the genetic background of this system in WG. We screened variations in the genes encoding leptin, ghrelin and their receptors, the leptin receptor (LEPR) and the growth hormone secretagogue receptor (GHSR). Three single nucleotide polymorphisms (SNPs) in each gene region were analysed in 460 German WG cases and 878 ethnically matched healthy controls. A three-SNP haplotype of GHSR was significantly associated with WG [P = 0.0067; corrected P-value (P(c)) = 0.026; odds ratio (OR) = 1.30; 95% CI 1.08, 1.57], as was one non-synonymous SNP in LEPR (Lys656Asn, P = 0.0034; P(c) = 0.013; OR = 0.72; 95% CI 0.58, 0.90). These four SNPs were re-analysed in independent cohorts of 226 German WG cases and 519 controls. While the GHSR association was not confirmed, allele frequencies of the LEPR SNP were virtually identical to those from the initial cohorts. Analysis of this SNP in the combined WG and control panels revealed a significant association of the LEPR 656Lys allele with WG (P = 0.00032; P(c) = 0.0013; OR = 0.72; 95% CI 0.60, 0.86). Remarkably, the Lys656Asn SNP showed contrasting allele distribution in two cohorts of 108 and 88 German cases diagnosed with Churg-Strauss syndrome (CSS, combined P = 0.0067; OR = 1.41; 95% CI 1.10, 1.81), whereas identical allele frequencies were revealed when comparing British WG and microscopic polyangiitis cases. While GHSR has to be further evaluated, these data provide profound evidence for an association of the LEPR Lys656Asn SNP with AAV, resulting in opposing effects in WG and CSS.

  19. Hemorragia alveolar maciça como manifestação inicial de poliangeíte microscópica Diffuse alveolar hemorrhage as initial manifestation of microscopic polyangiitis

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    Cristiano Barbosa Campanholo

    2007-02-01

    Full Text Available Hemorragia alveolar (HA é uma manifestação clínica com alta taxa de mortalidade que deve ser investigada, reconhecida e estabilizada. Causas possíveis para a HA incluem infecções respiratórias ou sistêmicas, malformações arteriovenosas, estenose mitral, discrasias sangüíneas e doenças auto-imunes, como o lúpus eritematoso sistêmico (LES, a síndrome de Goodpasture e as vasculites sistêmicas primárias, principalmente aquelas associadas aos anticorpos anticitoplasma de neutrófilos (Anca, como a granulomatose de Wegener, síndrome de Churg-Strauss e a poliangeíte microscópica. Relatamos o caso de uma paciente jovem que apresentou quadro grave de HA necessitando ventilação mecânica assistida com pressão expiratória final positiva (Peep. Na ausência de evidências de infecção, discrasias sangüíneas ou malformações arteriovenosas, a paciente foi submetida à corticoterapia e à imunossupressão, com controle satisfatório da atividade da doença, que persiste após 24 meses de seguimento. Os dados clínicos, laboratoriais e histopatológicos permitiram estabelecer o diagnóstico de poliangeíte microscópica.Alveolar hemorrhage (AH is a clinical manifestation with high mortality rate that must be promptly investigated, recognized and stabilized. Causes of AH include systemic and respiratory tract infections, arterio-venous malformations, blood dyscrasias and autoimmune diseases such as systemic lupus erithematosus, Goodpasture syndrome and primary systemic vasculitis, specially the antineutrophil cytoplasmic antibodies-associated vasculitis such as Wegener’s granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. We report the case of a young female patient who developed severe AH necessitating orotracheal intubation and mechanic assisted ventilation with positive end expiratory pressure. Since no evidence of infection, malformation or blood dyscrasia was found, immediate therapy with glucocorticoids and

  20. Clinicopathological study of vasculitic peripheral neuropathy

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    Rong-fang DONG

    2014-06-01

    anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis and 2 patients as immuno-mediated interstitial lung disease (ILD], and 3 patients were diagnosed as secondary systemic vasculitis. The other 3 patients were diagnosed as nonsystemic vasculitic periphral neuropathy(NSVPN. Conclusions The pathological features of VPN showed that the axonal degeneration was more serious than the myelin lesions because axons were more vulnerable to ischemia than Schwann cells, perineurial cells and fibroblasts. The pathology of VPN are diverse, which are associated with the disease progression and treatment, and therefore the hemo-immunologic parameters, nerve and muscle biopsy should be checked when the VPN is suspected. doi: 10.3969/j.issn.1672-6731.2014.06.011

  1. Binding of plasma proteins to titanium dioxide nanotubes with different diameters

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    Kulkarni M

    2015-02-01

    Full Text Available Mukta Kulkarni,1,* Ajda Flašker,1,* Maruša Lokar,1 Katjuša Mrak-Poljšak,2 Anca Mazare,3 Andrej Artenjak,4 Saša Čučnik,2 Slavko Kralj,5 Aljaž Velikonja,1 Patrik Schmuki,3 Veronika Kralj-Iglič,6 Snezna Sodin-Semrl,2,7 Aleš Iglič11Laboratory of Biophysics, Faculty of Electrical Engineering, University of Ljubljana, Ljubljana, Slovenia; 2Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia; 3Department of Materials Science and Engineering, University of Erlangen Nuremberg, Erlangen, Germany; 4Sandoz Biopharmaceuticals Mengeš, Lek Pharmaceuticals dd, Menges, Slovenia; 5Department for Materials Synthesis, Institute Jožef Stefan (IJS, Ljubljana, Slovenia; 6Faculty of Health Studies, University of Ljubljana, Ljubljana, Slovenia; 7Faculty of Mathematics, Natural Science and Information Technology, University of Primorska, Koper, Slovenia *These authors contributed equally to this workAbstract: Titanium and titanium alloys are considered to be one of the most applicable materials in medical devices because of their suitable properties, most importantly high corrosion resistance and the specific combination of strength with biocompatibility. In order to improve the biocompatibility of titanium surfaces, the current report initially focuses on specifying the topography of titanium dioxide (TiO2 nanotubes (NTs by electrochemical anodization. The zeta potential (ζ-potential of NTs showed a negative value and confirmed the agreement between the measured and theoretically predicted dependence of ζ-potential on salt concentration, whereby the absolute value of ζ-potential diminished with increasing salt concentrations. We investigated binding of various plasma proteins with different sizes and charges using the bicinchoninic acid assay and immunofluorescence microscopy. Results showed effective and comparatively higher protein binding to NTs with 100 nm diameters (compared to 50 or 15 nm. We also showed a dose

  2. Cutaneous factitia in elderly patients: alarm signal for psychiatric disorders

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    Chiriac A

    2014-03-01

    Full Text Available Anca Chiriac,1 Liliana Foia,2 Cristina Birsan,1 Ancuta Goriuc,2 Caius Solovan3 1Department of Dermatology, Nicolina Medical Center, Iaşi, Romania; 2Surgical Department, Grigore T Popa University of Medicine and Pharmacy, Iaşi, Romania; 3Department of Dermatology, Victor Babeş University of Medicine and Pharmacy, Timişoara, Romania Background: The factitious disorders, more commonly known in daily practice as pathomimia, are expressed in dermatology units by skin lesions induced voluntarily by the patient, in order to draw attention of the medical staff and/or the family members. The disorder is often challenging to diagnose and even more difficult to document in front of the patient or relatives. It represents a challenge for the physician, and any attempt at treatment may be followed by recurrence of the self-mutilation. This paper describes two cases of pathomimia diagnosed by dermatologists and treated in a psychiatry unit, highlighting the importance of collaboration in these situations. Patients and methods: Two case reports, describing old female patients with pathomimia, hospitalized in a department of dermatology for bizarre skin lesions. Results: The first case was a 77-year-old female with unknown psychiatric problems and atrophic skin lesions on the face, self-induced for many months, with multiple hospitalizations in dermatology units, with no response to different therapeutic patterns, and full recovery after psychiatric treatment for a major depressive syndrome. The second case was a 61-year-old female patient with disseminated atrophic scars on the face, trunk, and limbs. She raised our interest because of possible psychiatric issues, as she had attempted to commit suicide. The prescription of antidepressants led to a significant clinical improvement. Conclusion: These cases indicate that a real psychiatric disease may be recorded in patients suffering from pathomimia. Therefore, complete psychiatric evaluation in order to

  3. Necrotizing RPGN with linear anti IgG deposits in a patient with history of granulomatosis with polyangiitis: a case report

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    Parekh N

    2014-11-01

    pulmonary hemorrhage. In this case, since the patient did not have detectable circulating anti-GBM antibody, the decision was made not to proceed with plasmapheresis. The patient was treated with a standard immunosuppressive regimen consisting of prednisone and cyclophosphamide with partial renal recovery at 2 months. Keywords: Necrotizing RPGN, Anti-GBM disease, GPA, ANCA - associated vasculitis, dual antibody-positive disease

  4. Parámetros y valores genéticos para características de composición corporal, área de ojo del lomo y grasa dorsal medidos mediante ultrasonido en la raza Brahman

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    A. Jiménez

    2010-12-01

    Full Text Available El ganado Brahman en Colombia es el de mayor participación como raza pura paraproducción de carne, y tiene gran influencia en el ganado comercial. Hasta el momento,Asocebu ha realizado evaluaciones genéticas para características de crecimiento,pero aún no se han realizado para características de la canal. El objetivo de este trabajofue determinar parámetros genéticos (heredabilidades y correlaciones, y valores genéticos(DEP para área de ojo del lomo (AOL, grasa dorsal (GD, profundidad delmúsculo glúteo medio (PMGM y grasa del anca (GA. Fueron medidos por medio deultrasonido un total de 934 animales puros, hijos de 164 toros que se encontraban enun rango de edad de 15 a 18 meses. Para los análisis se crearon grupos contemporáneosteniendo en cuenta la época, el sexo y el manejo alimenticio. Se realizó un análisis univariadousando un modelo reproductor, teniendo en cuenta el grupo contemporáneo(animales del mismo sexo, de la misma época y en el mismo manejo alimenticio, laedad fue tomada como covariable y la finca fue incluida en el modelo. Las heredabilidadesfueron 0,37 ± 0,11; 0,29 ± 0,10; 0,26 ± 0,10 y 0,11 ± 0,09 para AOL, GD,PMGM y GA respectivamente. Las DEP para AOL variaron de -2,84 a 3,43; para GDde -0,372 a 0,235; para PMGM de -0,187 a 0,235, y para GD de -0,176 a 0,298. Lascorrelaciones genéticas fueron positivas y altas indicando que la selección por musculaturano afecta el grado de acabado. Este trabajo mostró que en ganado Brahman puroexiste variación genética para las características medidas por ultrasonido relacionadascon la canal, lo cual permitirá tenerlas en cuenta en el programa de mejoramientogenético de la raza Brahman en Colombia.

  5. Demonstration of the proliferation marker Ki-67 in renal biopsies: correlation to clinical findings.

    Science.gov (United States)

    Nabokov, A; Waldherr, R; Ritz, E

    1997-07-01

    (beta = 0.72) and to interstitial fibrosis score (beta = 0.59). Interstitial PI was independently correlated to antineutrophil cytoplasmic antibodies (ANCA) titer (beta = 0.7) and interstitial fibrosis score (beta = 0.55), and it was the only one PI correlated to serum creatinine concentration (beta = 0.53). The independent association between interstitial PI and serum creatinine (beta = 0.64) was also found in IgAN. Proximal tubular PI was correlated to interstitial fibrosis score (beta = 0.59) and proteinuria (beta = 0.54). In MCD, high PI values were noted in proximal tubular cells (1.42) but not in glomeruli and the interstitium. In conclusion, assessment of proliferation activity by immunohistology provides additional information beyond conventional pathological techniques to evaluate disease activity and prognosis in renal biopsies.

  6. Síndrome de Churg Strauss: uma vasculite rara Churg strauss Syndrome: a rare vasculitis

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    Luciana Calvo Mardegan

    2004-04-01

    Full Text Available O objetivo deste trabalho é avaliar as características demográficas, clínicas, laboratoriais e histopatológica de pacientes com diagnóstico de síndrome de Churg Strauss (SCS acompanhados no ambulatório de vasculites do Hospital das Clínicas da Universidade Estadual de Campinas (HC/Unicamp. Foi realizado estudo retrospectivo dos prontuários dos pacientes com vasculite primária, classificados como SCS pelos critérios do American College of Rheumathology (ACR. Foram observados cinco pacientes com diagnóstico de SCS (M/F:3/2, caucasóides. A média de idade do início da doença foi 46 anos (40-55. A média de tempo de seguimento foi de 2,37 anos (0,25-6. Sintomas sistêmicos, asma, neuropatia periférica e lesão cutânea foram observados em todos os pacientes. Envolvimento cardiovascular, renal e de trato gastrintestinal estiveram presentes cada qual em um paciente. Todos os pacientes apresentaram eosinofilia (>10%,e o p-ANCA foi positivo nos três casos investigados. A radiografia de tórax revelou infiltrado pulmonar intersticial em dois casos; em um, associado à derrame pleural. Na histopatologia, evidências de eosinófilos extravascular foram encontradas nas biópsias cutâneas de 3/4 pacientes. Todos os pacientes foram tratados com prednisona. Em 3, houve a necessidade de associar-se imunossupressor. Nenhum paciente evoluiu a óbito. A SCS é uma patologia rara, com acometimento sistêmico, sendo a asma uma das principais e mais precoce manifestação. Todos os pacientes apresentaram sintomas sistêmicos, acometimento de pele e sistema nervoso periférico. Envolvimento renal, cardíaco e gastrointestinal foi observado, cada qual em um único paciente, diferindo de outras séries nas quais esses acometimentos são mais freqüentes. Embora seja uma patologia grave e de prognóstico reservado, observamos evolução favorável com tratamento.The aim of this report is to verify the demographic, clinical, laboratorial and

  7. Bioconversion of Coal: Hydrologic indicators of the extent of coal biodegradation under different redox conditions and coal maturity, Velenje Basin case study, Slovenia

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    Kanduč, Tjaša; Grassa, Fausto; Lazar, Jerneja; Jamnikar, Sergej; Zavšek, Simon; McIntosh, Jennifer

    2014-05-01

    Underground mining of coal and coal combustion for energy has significant environmental impacts. In order to reduce greenhouse gas emissions, other lower -carbon energy sources must be utilized. Coalbed methane (CBM) is an important source of relatively low-carbon energy. Approximately 20% of world's coalbed methane is microbial in origin (Bates et al., 2011). Interest in microbial CBM has increased recently due to the possibility of stimulating methanogenesis. Despite increasing interest, the hydrogeochemical conditions and mechanisms for biodegradation of coal and microbial methane production are poorly understood. This project aims to examine geochemical characteristics of coalbed groundwater and coalbed gases in order to constrain biogeochemical processes to better understand the entire process of coal biodegradation of coal to coalbed gases. A better understanding of geochemical processes in CBM areas may potentially lead to sustainable stimulation of microbial methanogenesis at economical rates. Natural analogue studies of carbon dioxide occurring in the subsurface have the potential to yield insights into mechanisms of carbon dioxide storage over geological time scales (Li et al., 2013). In order to explore redox processes related to methanogenesis and determine ideal conditions under which microbial degradation of coal is likely to occur, this study utilizes groundwater and coalbed gas samples from Velenje Basin. Determination of the concentrations of methane, carbondioxide, nitrogen, oxygen, argon was performed with homemade NIER mass spectrometer. Isotopic composition of carbon dioxide, isotopic composition of methane, isotopic composition of deuterium in methane was determined with Europa-Scientific IRMS with an ANCA-TG preparation module and Thermo Delta XP GC-TC/CF-IRMS coupled to a TRACE GC analyzer. Total alkalinity of groundwater was measured by Gran titration. Major cations were analyzed by ICP-OES and anions by IC method. Isotopic composition of

  8. Jovan Hadžić's civil procedure code draft (1845

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    Stanković Uroš

    2013-01-01

    postupanju i blagodejanijama', probably related to bankruptcy proceedings and arbitration tribunal. Within the third part, one can find solely two short titles encompassing provisions on judge and attorney's service. The Prince forwarded consigned draft to State Council. Thereafter the Council established the Commission to revise the Draft on August 20th 1845. The Commission was made up of Stojan Simić, president of the Council (as the chairman of the Commission, state counselors Lazar Arsenijević Batalaka and Stojan Jovanović, the Council's Secretary Jovan Marinović, Director of Prince's Office Aleksa Janković, professor of law Maksim Simonović, secretary of Appellate Court Jakov Dimšić, secretary of Archdiocese Consistory Court Vasilije Lazić and secretary of Ministry of Justice Pavle Popović. The Commission made numerous alterations in the original text of the draft. Several titles were repositioned (settlement in bankruptcy proceedings, special proceedings and advantages, settlement in regular litigations and unnamed first title of the second part, probably referring to recusal. Some other remained in the same place, but were completely altered (rules on peace courts and unnamed fourth title in the first part, provisions on liquidation and arbitration tribunals and the whole third part. Rules on enforcement of judgements were modified exclusive of several provisions. The Commission also passed a multitude of remarks on contents and style of the draft's articles, whereas the first-mentioned were far more frequent. Only a few provisions did not undergo any modifications. By reason of considerable corrections had been made, the Commission decided that the draft should be reproduced and confined that task to Maksim Simonović, its most qualified member. Howsoever, it turned out to be overcomplicated due to excessive changes and inability to reproduce some titles in a fashion to regulate respective matters detailedly enough. Consequently, the Commission ordered

  9. Estates of Chilandar's pyrgos Hrusia (Chryseia in Kosovo and Metohija in the middle ages

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    Zarković Božidar V.

    2016-01-01

    Kosovo and included the Church of the Holy Virgin in Lipljan, the village Slovinje with 23 families of dependent people, and the revenues of the market in Lipljan, when it was organized to celebrate the days of the church. A charter describes the boundaries of the village, so we know where that land property was located. It was on the East of Lipljan, between the villages of Gušterica and Smoluša. These villages still exist with the same names. The second part of the estate was located in the North of Metohija, in the area of Metohisjki Podgor. It included half of a vineyard, a piary, a meadow and several large parts of land which were located between the villages of Žakovo, Suvo Grlo, Banje, Rudnik and Kostrc. Since all these villages still exist with the same names, it is easy to determine where the slump was. This slump was managed by the oikonomos, which means it was unique. These estates were confirmed by the Emperor Dušan in the General Chilandar charter of 1348. This ruler gifted the pyrgos with 1000 perpera of silver from Novo Brdo. Pyrgos fell into problems after the Battle of Maritsa in 1371, when the estates south of the Šar mountains fell under the rule of the Turks. The situation worsened when Prince Lazar took possession of Lipljan, because the Pyrgos' monks did not comply with the agreement that they had to build defenses against the Turks, and stole the money and gifts the Prince had given them for this aim. The danger which threatened united the monks of the monastery and the pyrgos, and after the Battle of Kosovo, together they sake contribution from Princess Milica and her sons Stefan and Vuk. She accepted and donated villages in the area of Morava in the South-East of Kosovo. The documents does not provide information about what happened to the estates of pyrgos Chryseia in the fifteenth century. We think that they survived while the Lazarevic and Brankovic ruled in these parts, up to the year 1455.

  10. Selected Abstracts of the 2nd Congress of joint European Neonatal Societies (jENS 2017; Venice (Italy; October 31-November 4, 2017; Session "Neonatal Hematology and Bilirubin"

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    --- Various Authors

    2017-10-01

    ABS 8. PHYSIOLOGIC FREE BILIRUBIN REDISTRIBUTES L1 CELL ADHESION MOLECULE (L1 IN LIPID RAFTS • N. Tang, M. He, J.F. Watchko, C.F. BearerABS 9. RETICULOCYTE HEMOGLOBIN CONTENT AS AN EARLY INDEX OF IRON DEFICIENCY IN NEONATAL PERIOD • P. Karagianni, E. Chatzitoliou, V. Antari, M. Lithoxopoulou, G. Mitsiakos, C. Tsakalidis, A. Kioumi, V. SoubasiABS 10. PREDICTION OF MAJOR BLEEDING IN EXTREMELY LOW BIRTH WEIGHT INFANTS (< 1,000 g BY SEQUENTIAL COAGULATION MONITORING • M. Thanhaeuser, C. Binder, M. Kornsteiner-Krenn, U. Derhaschnig, B. Jilma, A. Repa, A. Berger, N. HaidenABS 11. IV FLUID SUPPLEMENTATION IN SEVERE NEONATAL HYPERBILIRUBINEMIA: YES OR NO • M.T. Saleh, S. El Doory, S. Saleh, Mel. HalikABS 12. RIGHT SUBCLAVIAN ARTERY THROMBOSIS. A CASE REPORT • G. Uclés, T. Fuentes, R. Villarino, M. González-Valcárcel, A. MartínezABS 13. RISK FACTORS FOR PORTAL VENOUS THROMBOSIS IN NEWBORNS: A RET­RO­SPECTIVE STUDY • A. Zanin, M. Colella, C. Farnoux, S. Soudee, M. Leport, R. Stern, O. Bequet, M. Alison, V. Biran, O. BaudABS 14. COMPARISONS BETWEEN TRANSCUTANEOUS BILIRUBINOMETRY, POINT-OF-CARE WHOLE BLOOD BILIRUBIN AND TOTAL PLASMA BILIRUBIN MEASUREMENT IN NEONATES • K. Palmkvist Kaijser, C. Backman Johansson, K. Hemse Nojd, C. Bemgard, K. Kjellson, G. MarchiniABS 15. RELATIONSHIP BETWEEN PLASMA LEVELS OF BILIRUBIN AND OXIDANT/ANTIOXIDANT STATUS IN THE EARLY NEONATAL PERIOD IN PRETERM INFANTS • G.I. Zoutkamp, K. Suzuki, C.H.P. van den AkkerABS 16. RELATIONSHIP BETWEEN LEUKOCYTES AND PLATELETS AND PREMATURE RETINOPATHY • V. Filip, C. LazarABS 17. CONGENITAL HEPATOCELLULAR CARCI­NO­MA ASSOCIATED WITH NEONATAL HEMO­CHROMATOSIS • P. Francalanci, C. Grimaldi, M.C. Saffioti, R. Angelico, R. Boldrini, F. CalleaABS 18. INVESTIGATION OF THE RELATIONSHIP BETWEEN UMBILICAL CORD BLOOD RMI LEVEL AND PERINATAL EVENTS • Z.E. Erdem Dursun, N. Guzoglu, D. AliefendiogluABS 19. OUTCOME OF BABIES WITH SERUM BILIRUBIN LEVEL OF GREATER THAN 400 MICROMOL/L • R. Prasad, S

  11. When Coke Is Not Hydrating

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    Mohammed Bahaa Aldeen MD

    2014-09-01

    Full Text Available A 47-year-old African American man was admitted with 4 days of back pain, nausea and vomiting, and low urine output. There was no history of fever, dysuria, frequency, hesitancy, viral symptoms, trauma, rash, or constipation. Despite his past medical history of hypertension, diabetes mellitus, and hyperlipidemia he denied taking any medications for 18 months, including nonsteroidal anti-inflammatory drugs, acetaminophen, or antacids. He denied smoking and alcohol but admitted to cocaine use. No significant FH. Physical examination results were as follows: BP 235/125 mm Hg, heart rate 90 beats/min, temperature 98°F, O2 saturation normal; lungs and heart normal, abdomen soft but bilateral costovertebral angle tenderness. Neurological examination was normal. Laboratory tests yielded the following results: creatinine (Cr 10.5 mg/dL (1.2 mg/dL in 2010, blood urea nitrogen 63 mg/dL, glucose 151 mg/dL, Ca 9.4 mg/dL, PO4 6.1 mg/dL, Hgb 15 g/dL, white blood cells (WBC 9100, platelets 167 000, amylase/lipase normal, aspartate aminotransferase/alanine aminotransferase (AST/ALT normal, bilirubin 1.4 mg/dL, alkaline phosphatase 39 IU/L, creatine phosphokinase 127 µg/L. Hepatic panel, C- and P-ANCA (cytoplasmic– and perinuclear–antineutrophil cytoplasm antibodies, respectively, anti-GBM (anti–glomerular basement membrane, antimyeloperoxidase, antinuclear antibody, and Helicobacter pylori were all negative. C3, C4 normal, urinalysis: 2+ blood, no white blood cells or eosinophils, no casts, no albumin, negative for nitrate/leukocyte esterase and bacteria. Imaging: chest radiograph, abdominal radiograph, computed tomography of the abdomen, electrocardiography, and transthoracic echocardiography were all normal. Course. The patient’s urine output declined from 700 to 400 cm3/d and the on third day he required hemodialysis with Cr 14 mg/dL. Renal biopsy showed typical findings of interstitial nephritis. The patient was dialyzed for 10 days and responded

  12. Introduction pages

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    Radu E. Sestras

    2015-09-01

    Full Text Available Introduction Pages and Table of Contents Research ArticlesInsulin Requirements in Relation to Insulin Pump Indications in Type 1 DiabetesPDFGabriela GHIMPEŢEANU,\tSilvia Ş. IANCU,\tGabriela ROMAN,\tAnca M. ALIONESCU259-263Comparative Antibacterial Efficacy of Vitellaria paradoxa (Shea Butter Tree Extracts Against Some Clinical Bacterial IsolatesPDFKamoldeen Abiodun AJIJOLAKEWU,\tFola Jose AWARUN264-268A Murine Effort Model for Studying the Influence of Trichinella on Muscular Activity of MicePDFIonut MARIAN,\tCălin Mircea GHERMAN,\tAndrei Daniel MIHALCA269-271Prevalence and Antibiogram of Generic Extended-Spectrum β-Lactam-Resistant Enterobacteria in Healthy PigsPDFIfeoma Chinyere UGWU,\tMadubuike Umunna ANYANWU,\tChidozie Clifford UGWU,\tOgbonna Wilfred UGWUANYI272-280Index of Relative Importance of the Dietary Proportions of Sloth Bear (Melursus ursinus in Semi-Arid RegionPDFTana P. MEWADA281-288Bioaccumulation Potentials of Momordica charantia L. Medicinal Plant Grown in Lead Polluted Soil under Organic Fertilizer AmendmentPDFOjo Michael OSENI,\tOmotola Esther DADA,\tAdekunle Ajayi ADELUSI289-294Induced Chitinase and Chitosanase Activities in Turmeric Plants by Application of β-D-Glucan NanoparticlesPDFSathiyanarayanan ANUSUYA,\tMuthukrishnan SATHIYABAMA295-298Present or Absent? About a Threatened Fern, Asplenium adulterinum Milde, in South-Eastern Carpathians (RomaniaPDFAttila BARTÓK,\tIrina IRIMIA299-307Comparative Root and Stem Anatomy of Four Rare Onobrychis Mill. (Fabaceae Taxa Endemic in TurkeyPDFMehmet TEKİN,\tGülden YILMAZ308-312Propagation of Threatened Nepenthes khasiana: Methods and PrecautionsPDFJibankumar S. KHURAIJAM,\tRup K. ROY313-315Alleviate Seed Ageing Effects in Silybum marianum by Application of Hormone Seed PrimingPDFSeyed Ata SIADAT,\tSeyed Amir MOOSAVI,\tMehran SHARAFIZADEH316-321The Effect of Halopriming and Salicylic Acid on the Germination of Fenugreek (Trigonella foenum-graecum under Different Cadmium

  13. Silver segregation in Ag/a-C nanocomposite coatings for potential application as antibacterial surfaces

    Science.gov (United States)

    Manninen, Noora Kristiina Alves de Sousa

    sinovial, presente nas articulacoes da anca, o comportamento tribologico e semelhante aos revestimentos a-C.

  14. Monoclonal gammopathy in rheumatic diseases.

    Science.gov (United States)

    Yang, Yue; Chen, Long; Jia, Yuan; Liu, Yang; Wen, Lei; Liang, Yaoxian; An, Yuan; Chen, Shi; Su, Yin; Li, Zhanguo

    2018-07-01

    To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed. MG was observed in patients with SS, rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, primary biliary cirrhosis, polymyositis, hypomyopathic dermatomyositis, psoriatic arthritis, ANCA-associated vasculitis, polyarteritis nodosa, and polymyalgia rheumatic, with SS the most frequent type. Serum M protein was detected in 37 patients. The monoclonal bands identified in serum were 16 IgG (5 κ, 11 λ), 11 IgA (6 κ, 5 λ), 6 IgM (5 κ, 1 λ), and 4 free λ chains. M components were observed in urine in the other 4 patients. High ESR, albumin/globulin inversion, rheumatoid factor positivity, hypergammaglobulinemia, and hypocomplementemia were common features, presented in more than half of the 41 patients. Patients with pSS, when complicated with MG, showed a higher rate of abnormal urine NAG (71.4 vs 15.8%, P = 0.025), higher levels of ESR [55.0 (53.5) mm/h vs 21.0 (31.8) mm/h, P = 0.001], ESSDAI [26.0 (25.0) vs 12.0 (9.0), P = 0.006], and ClinESSDAI scores [24.0 (25.0) vs 10.5 (10.0), P = 0.011]. Multivariate analysis revealed that the disease activity, assessed by either ESSDAI [adjusted OR 1.127 (95%CI 1.015-1.251), P = 0.025] or ClinESSDAI [adjusted OR 1.121 (95%CI 1.011-1.242), P = 0.030], was the only independent risk factor for the presence of MG. During the follow-up, 2 patients had transient serum M protein, 2 had isotype

  15. Diagnostic pitfalls in a young Romanian ranger with an acute psychotic episode

    Directory of Open Access Journals (Sweden)

    Nagy EE

    2016-05-01

    Full Text Available Elöd Ernö Nagy,1,2 Attila Rácz,3 Edit Urbán,4 Gabriella Terhes,4 Timea Berki,5 Emöke Horváth,6 Anca M Georgescu,7 Iringó E Zaharia-Kézdi71Department of Pharmaceutical Biochemistry, University of Medicine and Pharmacy of Târgu-Mureş, 2Laboratory of Medical Analysis, Mures Clinical County Hospital, 3II. Psychiatry Clinic, Mures Clinical County Hospital, Târgu Mureş, Romania; 4Faculty of Medicine, Institute of Clinical Microbiology, University of Szeged, Szeged, 5Faculty of Medicine, Institute of Immunology and Biotechnology, University of Pécs, Pécs, Hungary; 6Department of Pathology, 7I. Clinic of Infectious Disease, University of Medicine and Pharmacy, Târgu Mureş, RomaniaAbstract: The identification and distinction of the pathological conditions underlying acute psychosis are often challenging. We present the case of a 35-year-old ranger who had no history of acute or chronic infectious disease or any previous neuropsychiatric symptoms. He arrived at the Psychiatry Clinic and was admitted as an emergency case, displaying bizarre behavior, hallucinations, paranoid ideation, and delusional faults. These symptoms had first appeared 7 days earlier. An objective examination revealed abnormalities of behavior, anxiety, visual hallucinations, choreiform, and tic-like facial movements. After the administration of neuroleptic and antidepressant treatment, he showed an initial improvement, but on day 10 entered into a severe catatonic state with signs of meningeal irritation and was transferred to the intensive care unit. An electroencephalogram showed diffuse irritative changes, raising the possibility of encephalitis. Taking into consideration the overt occupational risk, Borrelia antibody tests were prescribed and highly positive immunoglobulin (IgM and IgG titers were obtained from serum, along with IgG and antibody index positivity in cerebrospinal fluid. In parallel, anti-N-methyl-D-aspartate receptor antibodies and a whole

  16. Central nervous system involvement in patients with HCV-related cryoglobulinemia: review and a case report

    Directory of Open Access Journals (Sweden)

    B. Canesi

    2011-09-01

    % cryoglobulins were present, HCV antibody and HCV-RNA (type 2a-2c were positive. Cryoglobulins were never typed, because they disappeared after plasma exchanges. Liver enzymes, renal function and findings on cerebrospinal fluid were normal. Cerebral CT and MRI were also normal. Antinuclear antibodies, anti nDNA antibodies, antiphospholipid antibodies, lupus anticoagulant, ANCA, Lyme disease serology, complete tests for thrombophilia were negative. Bone aspiration was normal. The patient, in coma, was treated with two plasma exchanges. During the first treatment she recovered consciousness. Prednisone (1 mg/Kg/day and cyclophosphamide (400 mg iv for three days were added. After a week two plasma exchanges were performed again. Liver enzymes and rheumatoid factor were analyzed monthly for six months and than every two months for another six month period up to the present. Liver enzymes were always normal, rheumatoid factor was always at a lower level than the first evaluation (now it’s 311 U/ml. At present she is taking Prednisone 5 mg once a day, neurologic syntoms are absent and neurologic examination is normal. Discussion: We can conclude that: central neurologic involvement may be the clinical presentation of HCV infection and mixed cryoglobulinemia. HCV serologic tests and cryoglobulins should be considered in patient with encephalopathy of non-obvious cause; plasma exchange is the treatment of choice in acute severe forms; in some patients HCV could involve directly CNS, even in the absence of cryoglobulin production.

  17. Anestesia para cirurgia ortopédica em criança com susceptibilidade à hipertermia maligna: relato de caso Anestesia para cirugía ortopédica en niño con susceptibilidad a la hipertermia maligna: relato de caso Anesthesia for orthopedic surgery in a child susceptible to malignant hyperthermia: case report

    Directory of Open Access Journals (Sweden)

    Renato Santiago Gomez

    2003-02-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: Hipertermia maligna é uma miopatia autossômica dominante desencadeada por anestésicos inalatórios e bloqueadores neuromusculares, como halotano e succinilcolina, causando aumento da temperatura que pode ser fatal sem o tratamento imediato. O objetivo deste relato é descrever a conduta anestésica em uma criança susceptível à hipertermia maligna que foi submetida a procedimento cirúrgico ortopédico. RELATO DO CASO: Paciente do sexo feminino, com 3 anos, portadora de luxação congênita do quadril e susceptibilidade à hipertermia maligna, conforme história de anestesia anterior, foi submetida à correção cirúrgica ortopédica sob anestesia geral, com propofol e fentanil, associada à anestesia peridural lombar. A temperatura da paciente foi monitorizada continuamente durante a cirurgia e no período pós-operatório. A paciente apresentou recuperação pós-operatória sem intercorrências e recebeu alta hospitalar após cinco dias. CONCLUSÕES: A combinação de anestesia regional e venosa para o procedimento cirúrgico proposto em paciente com susceptibilidade à hipertermia maligna permitiu a condução anestésica com segurança.JUSTIFICATIVA Y OBJETIVOS: Hipertermia maligna es una miopatia autosómica dominante desencadenada por anestésicos inhalatorios y bloqueadores neuromusculares, como halotano y succinilcolina, causando aumento de la temperatura que puede ser fatal sin el tratamiento inmediato. El objetivo de este relato es describir la conducta anestésica en un niño susceptible a la hipertermia maligna que fue sometida a procedimiento quirúrgico ortopédico. RELATO DEL CASO: Niño, del sexo femenino, con 3 años, portadora de luxación congénita del cuadril (anca y susceptibilidad a la hipertermia maligna, conforme historia de anestesia anterior, fue sometida a la corrección quirúrgica ortopédica bajo anestesia general, con propofol y fentanil, asociada a anestesia peridural lumbar. La

  18. First Paleomagnetic Map of the Easternmost Mediterranean Derived from Combined Geophysical-Geological Analysis

    Science.gov (United States)

    Eppelbaum, Lev; Katz, Youri

    2014-05-01

    he easternmost Mediterranean is a tectonically complex region evolving in the long term and located in the midst of the progressive Afro-Eurasian collision (e.g., Ben-Avraham, 1978; Khain, 1984). Both rift-oceanic systems and terrane belts are known to have been formed in this collision zone (Stampfli et al., 2013). Despite years of investigation, the geological-geophysical structure of the easternmost Mediterranean is not completely known. The formation of its modern complex structure is associated with the evolution of the Neotethys Ocean and its margins (e.g., Ben-Avraham and Ginzburg, 1990; Robertson et al., 1991; Ben-Avraham et al., 2002). The easternmost Mediterranean was formed during the initial phase of the Neotethys in the Early and Late Permian (Golonka and Ford, 2000; Stampfli et al., 2013). At present this block of the ocean crust situated in the northern part of the Sinai plate (Ben-Avraham, 1978; Eppelbaum et al., 2012, 2014) is object of our investigation. The easternmost Mediterranean region has attracted increasing attention in connection with the recent discoveries of significant hydrocarbon deposits in this region (e.g., Montadert et al., 2010; Schenk et al., 2010; Eppelbaum et al., 2012). For example, Schenk et al. (2010) consider that more than 4 trillion m3 of recoverable gas is available in the Levant Basin (which located in the central part of the easternmost Mediterranean). Currently seismic prospecting is the main tool used in hydrocarbon deposit discovery. However, even sophisticated seismic data analysis (e.g., Hall et al., 2005; Roberts and Peace, 2007; Gardosh et al., 2010; Marlow et al., 2011; Lazar et al., 2012), fails to identify the full complex structural-tectonic mosaic of this region, and more importantly, is unable to clarify its baffling complex tectonic evolution. This highlights the need for combined analysis of geophysical data associated with the paleomagnetic and paleobiogeographic conditions that can yield deep

  19. A fluid biopsy as investigating technology for the fluid phase of solid tumors

    Science.gov (United States)

    Kuhn, Peter; Bethel, Kelly

    2012-02-01

    comparisons between circulating tumor cells from prostate cancer patients and the prostate tumor derived LNCaP cell line' by Lazar et al elucidates differences between the two sample types. In this study, immunocytochemistry is used to compare the protein expression levels of total cytokeratin (CK) and androgen receptor (AR) in CTCs from patients with prostate cancer versus those of cell line cells and to determine what translational insights might be gained through the use of cell line data. This paper demonstrates that LNCaP cells are phenotypically different from CTCs, both in terms of cytomorphic features and relative expression levels of proteins. The ability to perform comparisons between cell lines and actual CTCs from cancer patients furthers our ability to translate experimental cell line data into understanding cancer in the human body and, thus, into predicting and influencing clinical outcomes. In the paper entitled 'High definition imaging of circulating tumor cells and associated cellular events in non-small cell lung cancer patients: a longitudinal analysis' by Nieva et al, the kinetics of CTCs over time is explored. Twenty eight patients with non-small cell lung cancer and hematogenously seeded metastasis were analyzed with multiple blood draws. We detected CTCs in 68% of analyzed samples and found a propensity for increased CTC detection as the disease progressed in individual patients. CTCs were present at a median concentration of 1.6 CTCs per milliliter of analyzed blood in the patient population. Higher numbers of detected CTCs were associated with an unfavorable prognosis. The paper entitled 'Fluid biopsy for circulating tumor cell identification in patients with early and late stage non-small cell lung cancer: a glimpse into lung cancer biology' by Wendel et al, describes similar numbers of CTCs in the bloodstream of patients with widely differing extent of disease at the time they were first diagnosed. Whether the tumors were found when they were small

  20. How Often do Giant Black Holes Become Hyperactive?

    Science.gov (United States)

    2010-12-01

    for field galaxies -- as some results have hinted -- but then decreased more rapidly, at some point the cluster fraction would be about equal to the field fraction. This may explain what is being seen in the local Universe. The Milky Way contains a supermassive black hole known as Sagittarius A* (Sgr A*, for short). Even though astronomers have witnessed some activity from Sgr A* using Chandra and other telescopes over the years, it has been at a very low level. If the Milky Way follows the trends seen in the ChaMP survey, Sgr A* should be about a billion times brighter in X-rays for roughly 1% of the remaining lifetime of the Sun. Such activity is likely to have been much more common in the distant past. If Sgr A* did become an AGN it wouldn't be a threat to life here on Earth, but it would give a spectacular show at X-ray and radio wavelengths. However, any planets that are much closer to the center of the Galaxy, or directly in the line of fire, would receive large and potentially damaging amounts of radiation. These results were published in the November 10th issue of the Astrophysical Journal. Other co-authors on the paper were Scott Anderson of the University of Washington, Anca Constantin from James Madison University, Tom Aldcroft and Dong-Woo Kim from Harvard-Smithsonian Center for Astrophysics and Wayne Barkhouse from the University of North Dakota. NASA's Marshall Space Flight Center in Huntsville, Ala., manages the Chandra program for NASA's Science Mission Directorate in Washington. The Smithsonian Astrophysical Observatory controls Chandra's science and flight operations from Cambridge, Mass. More information, including images and other multimedia, can be found at: http://chandra.harvard.edu and http://chandra.nasa.gov