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Sample records for vessel vasculitis involving

  1. Vessel involvement in giant cell arteritis : an imaging approach

    NARCIS (Netherlands)

    Holm, Pieter W.; Sandovici, Maria; Slart, Riemer H. J. A.; Glaudemans, Andor W. J. M.; Rutgers, Abraham; Brouwer, Elisabeth

    Vasculitis is classified based on the size of the involved vessels. The two major forms are small vessel vasculitis and large vessel vasculitis (LVV). Main forms of LVV are Takayasu arteritis, giant cell arteritis (GCA), isolated aortitis and chronic periaortitis. This manuscript will focus on GCA,

  2. Renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement

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    Slot, MC; Tervaert, JWC; Franssen, CFM; Stegeman, CA

    Background. Severe renal disease is a feature of anti-neutrophil cytoplasmic antibodies (ANCA)-associated small-vessel vasculitis. We evaluated patient and renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement at diagnosis during long-term

  3. The “other” vasculitis syndromes and kidney involvement

    Science.gov (United States)

    2009-01-01

    There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and/or are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of this group as it involves vessels of any size on both the arterial and venous side. In addition to renal vascular involvement, Behçet disease may involve the kidney through glomerulonephritis, secondary amyloidosis and, rarely, tubulointerstital involvement. Vasculitis secondary to infections, malignancy, and drugs are not common among children. However, vasculitis may be associated with a number of rheumatic diseases in childhood and the auto-inflammatory syndromes (periodic fever syndromes). Auto-inflammatory syndromes are diseases characterized by periodic attacks of clinical and laboratory inflammation. Studies carried out during the past decade have provided valuable information on the mechanism of inflammation and innate immunity in general. This group of vasculitides is associated with secondary amyloidosis of the kidney if not treated. Hypocomplementemic urticarial vasculitis is an interesting vasculitic disease with frequent kidney involvement. Here, we introduce the reader to the wide scope of these diseases; although rare, such diseases represent a challenge to the nephrologist. PMID:19898878

  4. Vasculites dos médios e grandes vasos Medium and large vessel vasculitis

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    Hebert Roberto Clivati Brandt

    2009-02-01

    Full Text Available As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal; pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas; e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia. Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis; medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases; and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia. This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.

  5. Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris

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    Motoko Kosaka

    2012-03-01

    Full Text Available We present the case of a 64-year-old woman who has suffered from pustulosis palmaris et plantaris for 10 years. At the first examination, many erythematous lesions with purpura, blood crusts, and blisters were present in the lower legs and dorsum of the feet. Painful swelling in the sternal region and dorsal pain were also noted. Elevation of the CRP and myogenic enzyme levels, and liver and renal dysfunctions were noted on blood testing. Histopathologically, leukocytoclastic vasculitis was noted in small blood vessels in the whole dermal layers, and deposition of IgM and C3 in the vascular wall was detected by the direct immunofluorescence techniques. Based on these findings, cutaneous small vessel vasculitis was diagnosed. Because the patient complained of a toothache during the clinical course, an X-ray examination was performed. On pantomography, a radicular cyst and apical periodontitis were noted. The tooth symptoms changed with exacerbation and remission of the skin symptoms. These findings indicate that odontogenic infection is very likely to be a cause of cutaneous small vessel vasculitis in a manner similar to pustulosis palmaris et plantaris.

  6. Determinants of Vessel Targeting in Vasculitis

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    Gary S. Hoffman

    2004-01-01

    Full Text Available Studies of autoimmune diseases have not yet elucidated why certain organs or vessels become the objects of injury while others are spared. This paper will explore the hypothesis that important differences exist in regions of the aorta that determine vulnerability to diseases, such as atherosclerosis, aortitis, giant cell arteritis and Takayasu's disease. The reader is invited to reassess; (1 whether the aorta is indeed a single homogeneous structure, and (2 whether the initial stage of aortitis (and indeed other diseases considered “autoimmune” may be primarily due to acquired alterations of substrate, that influence unique immune profiles, which by themselves may not be pathogenic. Disease susceptibility and patterns are influenced by many factors that are inborn and acquired. Examples include genetic background, gender, ethnicity, aging, prior and concomitant illnesses, habits, diet, toxin and environmental exposures. Studies of vascular diseases must assess how such variables may affect regional differences in endothelial cells, subendothelial matrix, vascular smooth muscle and the response of each to a variety of stimuli.

  7. [Hypocomplementaemic urticarial vasculitis with bullous lesions and pericardial involvement].

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    Kervarrec, T; Binois, R; Bléchet, C; Estève, É

    2015-10-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare disease involving urticarial cutaneous vasculitis, hypocomplementaemia and systemic manifestations. Pericardial involvement occurs in very rare cases. We report a case of HUVS associated with specific pericarditis and bullous lesions. A 63-year-old woman consulted for chronic urticaria that had appeared ten months earlier. Her skin lesions were associated with weight loss of 10 kg, deterioration of respiratory function and abdominal pain. Leukocytoclastic vasculitis was seen in the skin biopsy sample. Hypocomplementaemia and anti C1q antibodies were present and a diagnosis of HUVS was made. During hospitalisation, extensive compressive pericardial effusion was identified, and histological examination of the biopsy revealed specific pericardial lymphocytic vasculitis. During follow-up, four episodes of infectious pneumonitis were noted. Bullous skin lesions were also observed. HUVS is a disease caused by an antibody against C1q complement responsible for urticarial lesions and vasculitis antibodies. To our knowledge, there have been only five reports in the literature of pericardial injury associated with HUVS. In our case, histological examination of the pericardium demonstrated lymphocytic vasculitis. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  8. Vasculitis

    Science.gov (United States)

    ... nerves. It is crucial, of course, to prevent death and long-term disability from vasculitis. Yet, other issues often trouble patients. These include fatigue (feeling very tired), pain, arthritis, nose and sinus problems, ...

  9. Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV)

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    Pastuszczak, Maciej; Celińska-Löwenhoff, Magdalena; Sułowicz, Joanna; Wojas-Pelc, Anna; Musiał, Jacek

    2017-01-01

    Abstract Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation. The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers. Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed. A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5–176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2–45; P = 0.02). SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence. PMID:28328827

  10. Anti-neutrophil cytoplasmic antibody pathogenesis in small-vessel vasculitis: an update.

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    Gómez-Puerta, José A; Bosch, Xavier

    2009-11-01

    Vasculitides associated with serum positivity for anti-neutrophil cytoplasmic antibodies (ANCAs) that affect small- to medium-sized vessels are commonly known as ANCA-associated vasculitis (AAV) and include Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Evidence derived from both in vitro studies and recent animal models points to a pathogenic role of ANCAs in AAV. In 2002, the first in vivo breakthrough in the pathogenesis of ANCAs showed that mouse ANCAs against myeloperoxidase (MPO) led to intrinsic pauci-immune renal vasculitis in mice. In 2004, a report using both in vitro and in vivo studies proposed that proteinase 3 (PR3)-directed autoimmunity involved the complementary peptide of PR3 (cPR3), which is encoded by the antisense strand of the PR3 gene. The last breakthrough came in October 2008 with a previously undescribed molecular explanation for the origin and development of injury in pauci-immune renal vasculitis, with potential clinical implications. This report showed that infection by fimbriated bacteria may trigger cross-reactive autoimmunity to a previously characterized ANCA antigen, lysosomal membrane protein-2, which is contained in the same vesicles that harbor MPO and PR3. Infection by fimbriated bacteria resulted in the production of autoantibodies, which activated neutrophils and killed human microvascular endothelium in vitro and caused renal vasculitis in rats. Although the evidence for a pathogenic role of ANCAs, mainly MPO-ANCAs, is striking, various questions remain unanswered. Understanding the key pathogenic mechanisms of AAV may provide a safer, more rational therapeutic approach than the traditional (ie, corticosteroids and immunosuppressants) treatment strategy.

  11. Issues in trial design for ANCA-associated and large-vessel vasculitis.

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    Tarzi, Ruth M; Mason, Justin C; Pusey, Charles D

    2014-08-01

    Randomized clinical trials (RCTs) have informed the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, although challenges still exist. The evidence base for treating large-vessel vasculitis (LVV) is weaker, but initiatives to standardize diagnostic criteria and outcome measures, and to validate biomarkers in LVV, together with newly initiated RCTs should start to address this need. In this Perspectives, we discuss the prerequisites for RCTs in vasculitis, existing trial evidence, continuing unmet needs, potential therapeutic avenues to explore and considerations in the design of future trials.

  12. [Severe pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV)].

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    Raoufi, Mohammed; Laine, Mustapha; Amrani, Hicham Naji; Souhi, Hicham; Janah, Hicham; Elouazzani, Hanane; Rhorfi, Ismail Abderrahmane; Abid, Ahmed

    2016-01-01

    Pulmonary involvement in hypocomplementemic urticarial vasculitis (HUV) or Mac Duffie syndrome is extremely rare with a poor prognosis. We report the case of a 55-year-old female patient treated for HUV over a period of 20 years. The diagnosis was confirmed on the basis of urticarial lesions, ocular inflammation, positive C1q-p test by immunodiffusion, with low rate of C1q. The patient was treated with cycles of cyclophosphamide, corticoids and rituximab as she developed class III dyspnea (NYHA classification ). The clinico-radiological and functional assessment showed thoracic distension and severe obstructive pulmonary disease which found no significant improvement with systemic treatment Aerosol therapy was started and the patient had a marked clinical improvement. Pulmonary involvement in Mac Duffie hypocomplementemic urticarial vasculitis worsens the patient short-term vital prognosis. The knowledge of the different types of pulmonary involvement opens new therapeutic prospects.

  13. Assessment of disease activity in large-vessel vasculitis

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    Aydin, Sibel Z.; Direskeneli, Haner; Merkel, Peter A.

    2017-01-01

    Objective. To arrive at consensus for candidate outcomes for disease activity assessment in largevessel vasculitis (LVV) in clinical trials. Methods.A Delphi survey including 99 items was circulated among international experts for 3 rounds. Results. Fifty-seven items were accepted for both giant ...

  14. Varicella Zoster Virus and Large Vessel Vasculitis, the Absence of an Association

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    Gary W. Procop

    2017-06-01

    Full Text Available Objective: It is controversial whether microorganisms play a role in the pathogenesis of large and medium vessel vasculitides (eg, giant cell arteritis [GCA], Takayasu arteritis [TAK] and focal idiopathic aortitis [FIA]. Recent studies have reported the presence of Varicella Zoster Virus (VZV within formalin-fixed, paraffin-embedded temporal arteries and aortas of about three-quarters or more of patients with these conditions, and in a minority of controls. In a prospective study, we sought to confirm these findings using DNA extracted from vessels that were harvested under surgically aseptic conditions and snap frozen. Methods and Results: DNA samples extracted from 11 surgically sterile temporal arteries and 31 surgically sterile thoracic aortas were used in an attempt to identify the vessel-associated VZV genome. Two different validated PCR methods were used. Thirty-one thoracic aorta aneurysm specimens included biopsies from 8 patients with GCA, 2 from patients with TAK, 6 from patients with FIA, and 15 from patients without vasculitis, who had non-inflammatory aneurysms. Eleven temporal artery biopsies were collected from 5 patients with GCA and 6 controls. The presence of VZV was not identified in either the specimens from patients with large vessel vasculitis or from the controls. Conclusions: Using surgically sterile snap-frozen specimens, we were unable to confirm recent reports of the presence of VZV in either aortas or temporal arteries from patients with large vessel vasculitis or controls. Keywords: Aorta and temporal artery biopsies, Varicella Zoster Virus, Large Vessel Vasculitis

  15. Large Vessel Vasculitis Occurring in Rheumatoid Arthritis Patient under Anti-TNF Therapy

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    Valentina Cestelli

    2014-01-01

    Full Text Available Vasculitis is a heterogeneous group of disorders characterized by the presence of necrotic inflammatory phenomena and destruction of blood vessels. Vasculitis is classified as primary (idiopathic or secondary to infections, connective tissue diseases and drugs but can also be considered as a paraneoplastic phenomenon. Evidence shows that the increasing use of biological agents results in a growing number of reports of autoimmune diseases induced by these therapies. An inflammatory articular chronic disease such as rheumatoid arthritis may be complicated by extra-articular manifestations, such as cutaneous or systemic vasculitis. Herewith, we describe the case of a great vessels arteritis in a patient affected by rheumatoid arthritis in therapy with an anti-TNF agent (etanercept.

  16. A Case of Rheumatoid Vasculitis Involving Hepatic Artery in Early Rheumatoid Arthritis.

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    Lee, Ji Eun; Kim, In Je; Cho, Min Sun; Lee, Jisoo

    2017-07-01

    Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis. A 72-year-old woman who presented with polyarthralgia for 2 months was diagnosed with early RA. Since she had manifestations of livedo reticularis, and liver dysfunction which was atypical for RA patients, a percutaneous needle liver biopsy was performed revealing arteritis of a medium-sized hepatic artery. Extensive investigations did not reveal evidences of other systemic causes such as malignancy or systemic vasculitis. The patient was diagnosed with rheumatoid vasculitis involving hepatic arteries based on Bacon and Scott criteria for rheumatoid vasculitis. With high dose corticosteroid and cyclophosphamide induction and methotrexate and tacrolimus maintenance treatment, she was successfully recovered. Association of rheumatoid vasculitis at very early stages of the disease may represent an early aggressive form of RA. © 2017 The Korean Academy of Medical Sciences.

  17. Hypocomplementemic urticarial vasculitis complicated with diffuse alveolar hemorrhage: a case report and review of literature

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    Paola Gnerre

    2014-03-01

    Full Text Available Urticarial vasculitis is a small-vessel vasculitis characterized clinically by urticaria and microscopically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome is a rare subtype of urticarial vasculitis with organ involvement and persistent, acquired hypocomplementemia. Pulmonary involvement, usually seen as diffuse alveolar damage, is an uncommon complication and a significant cause of morbidity and mortality. Here we present a rare case of hypocomplementemic urticarial vasculitis syndrome complicated by diffuse alveolar damage and describe the beneficial effect of cyclophosphamide-dexamethasone.

  18. Imaging large vessel vasculitis with fully integrated PET/MRI: a pilot study

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    Einspieler, Ingo; Pyka, Thomas; Eiber, Matthias [Technische Universitaet Muenchen, Klinikum rechts der Isar, Department of Nuclear Medicine, Munich (Germany); Thuermel, Klaus; Wolfram, Sabine; Moog, Philipp [Technische Universitaet Muenchen, Klinikum rechts der Isar, Department of Nephrology, Munich (Germany); Reeps, Christian [Technische Universitaet Muenchen, Department of Vascular Surgery, Klinikum rechts der Isar, Munich (Germany); Essler, Markus [Rheinische Friedrich-Wilhelms-Universitaet, Department of Nuclear Medicine, Universitaetsklinikum Bonn, Bonn (Germany)

    2015-04-16

    The aim of this study was to evaluate the feasibility of hybrid [{sup 18}F]fluorodeoxyglucose (FDG) positron emission tomography (PET)/MRI in patients with large vessel vasculitis (LVV) by comparing visual and quantitative parameters to that of PET/CT. Furthermore, the value of PET/MRI in disease activity and extent of LVV was assessed. A total of 16 [{sup 18}F]FDG PET/MRI and 12 [{sup 18}F]-FDG PET/CT examinations were performed in 12 patients with LVV. MRI of the vessel wall by T1-weighted and T2-weighted sequences was used for anatomical localization of FDG uptake and identification of morphological changes associated with LVV. In addition, contrast-enhanced (CE) magnetic resonance angiography (MRA) was performed. The vascular FDG uptake in the vasculitis group was compared to a reference group of 16 patients using a four-point visual score. Visual scores and quantitative parameters [maximum standardized uptake value (SUV{sub max}) and target to background ratio (TBR)] were compared between PET/MRI and PET/CT. Furthermore, correlations between C-reactive protein (CRP) and quantitative PET results, as well the extent of vasculitis in PET, MRI/CE-MRA and combined PET/MRI, were analysed. TBRs, SUV{sub max} values and visual scores correlated well between PET/MRI and PET/CT (r = 0.92, r = 0.91; r = 0.84, p < 0.05). There was no significant difference between both modalities concerning SUV{sub max} measurements and visual scores. In PET/MRI, PET alone revealed abnormal FDG uptake in 86 vascular regions. MRI/CE-MRA indicated 49 vessel segments with morphological changes related to vasculitis, leading to a total number of 95 vasculitis regions in combination with PET. Strong and significant correlations between CRP and disease extent in PET alone (r = 0.75, p = 0.0067) and PET/MRI (r = 0.92, p < 0.0001) in contrast to MRI/CE-MRA only were observed. Regarding disease activity, no significant correlations were seen between quantitative PET results and CRP, although there

  19. Settings and artefacts relevant for Doppler ultrasound in large vessel vasculitis

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    Terslev, L; Diamantopoulos, A P; Døhn, U Møller

    2017-01-01

    Ultrasound is used increasingly for diagnosing large vessel vasculitis (LVV). The application of Doppler in LVV is very different from in arthritic conditions. This paper aims to explain the most important Doppler parameters, including spectral Doppler, and how the settings differ from those used...... in arthritic conditions and provide recommendations for optimal adjustments. This is addressed through relevant Doppler physics, focusing, for example, on the Doppler shift equation and how angle correction ensures correctly displayed blood velocity. Recommendations for optimal settings are given, focusing...

  20. Severe cholestatic hepatitis due to large vessel vasculitis: report of two cases.

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    Xu, Jason; Björnsson, Einar S; Sundaram, Vinay

    2015-12-14

    Giant cell arteritis (GCA) is a vasculitis of medium and large sized vessels that occurs most often in people >50 years of age with associated symptoms of fever, weight loss, headache and jaw claudication. Polymyalgia rheumatica (PMR), which is characterized by aching and stiffness in the shoulders, hip girdle, neck and torso, is intimately associated with GCA, and evidence suggests that GCA and PMR are two phases of the same disease. The occurrence of liver enzyme abnormalities in either of these conditions is rare. Furthermore, as these conditions occur most commonly in the elderly population who may be subject to polypharmacy, patients with elevated aminotransferases due to underlying GCA/PMR may mistakenly have their abnormal liver function tests attributed to drug-induced liver injury. Given the potential complications of these diseases if left untreated, including ischemic stroke and blindness, early recognition and treatment are critical. We report two patients who developed severe cholestatic liver enzyme elevation, which had been initially attributed to drug toxicity, but was ultimately caused by large vessel vasculitis, specifically GCA and PMR. © The Author(s) 2015. Published by Oxford University Press and the Digestive Science Publishing Co. Limited.

  1. Leg Pain From Periostitis as the First Clinical Manifestation of Large-Vessel Vasculitis: A Case Report.

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    Bogaert, Bert; Brys, Peter; Peers, Koen

    2017-07-03

    We report a case of a 47-year-old woman with isolated periostitis of the lower leg as a first manifestation of large-vessel vasculitis. The diagnosis was first suspected when a second magnetic resonance imaging of the lower leg approximately 4 months after onset of shin pain showed edema near intramuscular vessels. Isolated periostitis is a very rare skeletal manifestation of systemic vasculitis and easily could be misdiagnosed. This case demonstrates that maintaining a broad differential diagnosis for a common presenting complaint such as localized leg pain is imperative. To be determined. Copyright © 2017 American Academy of Physical Medicine and Rehabilitation. Published by Elsevier Inc. All rights reserved.

  2. Urticarial Vasculitis

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    ... Store Home / Resources / Forms of Vasculitis / Urticarial Vasculitis Urticarial Vasculitis Urticarial vasculitis is a form of vasculitis that ... of complements; it’s called hypocomplementemic vasculitis. What causes Urticarial Vasculitis? The cause of most cases of urticarial vasculitis ...

  3. Imaging of large vessel vasculitis with {sup 18}FDG PET: illusion or reality? A critical review of the literature data

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    Belhocine, Tarik; Vandevivere, Johan [Department of Nuclear Medicine, A.Z. Middelheim Hospital, 2020, Antwerp (Belgium); Blockmans, Daniel [Department of Internal Medicine, Gasthuisberg University Hospital, Leuven (Belgium); Hustinx, Roland [Department of Nuclear Medicine, University Hospital of Liege, Liege (Belgium); Mortelmans, Luc [Department of Nuclear Medicine, Gasthuisberg University Hospital, Leuven (Belgium)

    2003-09-01

    Fluorine-18 fluorodeoxyglucose positron emission tomography ({sup 18}FDG PET) plays a major role in the management of oncology patients. Owing to the singular properties of the glucose tracer, many patients suffering from non-malignant diseases such as inflammatory or infectious diseases may also derive clinical benefit from the appropriate use of metabolic imaging. Large vessel vasculitides such as giant cell arteritis and Takayasu arteritis are other examples that may potentially extend the field of {sup 18}FDG PET indications. The purpose of the present article is to assess the feasibility of metabolic imaging in vasculitis on the basis of the current literature data. In particular, the clinical context and the {sup 18}FDG imaging patterns seen in patients with large vessel vasculitis are analysed in order to identify potential indications for metabolic imaging. (orig.)

  4. Imaging of large vessel vasculitis with (18)FDG PET: illusion or reality? A critical review of the literature data.

    Science.gov (United States)

    Belhocine, Tarik; Blockmans, Daniel; Hustinx, Roland; Vandevivere, Johan; Mortelmans, Luc

    2003-09-01

    Fluorine-18 fluorodeoxyglucose positron emission tomography ((18)FDG PET) plays a major role in the management of oncology patients. Owing to the singular properties of the glucose tracer, many patients suffering from non-malignant diseases such as inflammatory or infectious diseases may also derive clinical benefit from the appropriate use of metabolic imaging. Large vessel vasculitides such as giant cell arteritis and Takayasu arteritis are other examples that may potentially extend the field of (18)FDG PET indications. The purpose of the present article is to assess the feasibility of metabolic imaging in vasculitis on the basis of the current literature data. In particular, the clinical context and the (18)FDG imaging patterns seen in patients with large vessel vasculitis are analysed in order to identify potential indications for metabolic imaging.

  5. 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

  6. Clinical significance of immunoglobulin A antiphospholipid antibodies: possible association with skin manifestations and small vessel vasculitis.

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    Tajima, C; Suzuki, Y; Mizushima, Y; Ichikawa, Y

    1998-09-01

    To clarify whether immunoglobulin A (IgA) antiphospholipid antibodies (aPL) are an independent risk factor for specific manifestations of collagen vascular diseases. We determined IgG, IgM, and IgA anticardiolipin antibodies (aCL) and lupus anticoagulant (LAC) in 77 patients with various collagen diseases. Fifty-four patients who had positive results for either or both antibody classes were compared to 23 patients with systemic lupus erythematosus who had none of these antibodies. The association between the antibodies and clinical manifestations (thrombosis, fetal loss, thrombocytopenia, biological false positive test for syphilis, cutaneous manifestations, central nervous system involvement, and renal involvement) was analyzed. Of 54 patients with aPL, 33 showed significantly high levels of IgA aCL. Among them, IgA aCL coexisted with other aCL isotypes or LAC in 24 patients. The 9 patients with IgA aCL alone frequently had vasculitis associated manifestations, although thrombotic events and recurrent fetal loss were rare. Multivariate linear regression analysis showed that IgA aCL were independently associated with thrombocytopenia, skin ulcers, chilblain lupus, and vasculitis. There was also an association between IgM aCL and skin ulcers or chilblain lupus. Clinical manifestations of patients with IgA aCL differ from those of patients with IgG aCL. Determination of all 3 aCL isotypes and LAC is important to assess the risk of specific clinical manifestations in patients with aPL.

  7. Settings and artefacts relevant for Doppler ultrasound in large vessel vasculitis.

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    Terslev, L; Diamantopoulos, A P; Døhn, U Møller; Schmidt, W A; Torp-Pedersen, S

    2017-07-20

    Ultrasound is used increasingly for diagnosing large vessel vasculitis (LVV). The application of Doppler in LVV is very different from in arthritic conditions. This paper aims to explain the most important Doppler parameters, including spectral Doppler, and how the settings differ from those used in arthritic conditions and provide recommendations for optimal adjustments. This is addressed through relevant Doppler physics, focusing, for example, on the Doppler shift equation and how angle correction ensures correctly displayed blood velocity. Recommendations for optimal settings are given, focusing especially on pulse repetition frequency (PRF), gain and Doppler frequency and how they impact on detection of flow. Doppler artefacts are inherent and may be affected by the adjustment of settings. The most important artefacts to be aware of, and to be able to eliminate or minimize, are random noise and blooming, aliasing and motion artefacts. Random noise and blooming artefacts can be eliminated by lowering the Doppler gain. Aliasing and motion artefacts occur when the PRF is set too low, and correct adjustment of the PRF is crucial. Some artefacts, like mirror and reverberation artefacts, cannot be eliminated and should therefore be recognised when they occur. The commonly encountered artefacts, their importance for image interpretation and how to adjust Doppler setting in order to eliminate or minimize them are explained thoroughly with imaging examples in this review.

  8. Small-vessel vasculitis surrounding an uninflamed temporal artery: a new diagnostic criterion for polymyalgia rheumatica?

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    Chatelain, Denis; Duhaut, Pierre; Loire, Robert; Bosshard, Sylvie; Pellet, Hélène; Piette, Jean-Charles; Sevestre, Henri; Ducroix, Jean-Pierre

    2008-08-01

    To assess the prevalence and clinical significance of small-vessel vasculitis (SVV) surrounding an uninflamed temporal artery (TA) in patients diagnosed as having giant cell (temporal) arteritis (GCA) and/or polymyalgia rheumatica (PMR). Patients with GCA and/or PMR (n = 490) were included in this multicenter prospective study. Slides of TA biopsy specimens were reviewed by 2 pathologists who were blinded with regard to clinical information. SVV was defined as aggregates of mononuclear inflammatory cells surrounding a capillary, distant from an uninflamed temporal artery. Clinical and biologic data of patients in the SVV group (n = 35) were compared with data of patients with biopsy-proven GCA (n = 280) and with negative TA biopsy findings (n = 175). SVV was diagnosed in 18 women and 17 men (mean +/- SD age 74.5 +/- 9.4 years). The group of patients with SVV had a higher proportion of men than in the entire GCA series, had systemic symptoms, headache, jaw claudication, and an abnormal temporal artery less frequently at clinical examination, but had symptoms of PMR more often than patients in the biopsy-proven GCA group (P = 2.6 x 10(-7), odds ratio 9.17 [95% confidence interval 3.44-24.4]). Levels of inflammation markers were significantly lower in the SVV group. Patients in the SVV group had fever less frequently than patients in the group with negative TA biopsy findings, but otherwise shared the same clinical (including PMR symptoms) and biologic features. Eighteen of the 94 patients with pure PMR (19%) had SVV. SVV is often neglected by pathologists, and appears to be strongly associated with PMR symptoms in patients with a clinical diagnosis of GCA and/or PMR. However, SVV as a new diagnostic criterion for PMR must be assessed in prospective studies.

  9. Urticarial vasculitis in an infant.

    Science.gov (United States)

    Koch, Phoebe E; Lazova, Rossitza; Rosen, James R; Antaya, Richard J

    2008-01-01

    Urticarial vasculitis (UV), a subtype of leukocytoclastic vasculitis, is a small vessel necrotizing vasculitis characterized by urticarial lesions and decreased serum complement. Primarily a disease of adult women, this immune complex disorder is seldom reported in children. Pathologic examination of skin lesions in patients with UV reveals leukocytoclastic vasculitis. UV is a frequent finding, particularly involving early components of the classical complement cascade C1q and C2 to C4. We report a 9-month-old male infant who presented with chronic urticaria of 7 months' duration that was unresponsive to conventional therapies for urticaria. His lesions appeared as erythematous wheals followed by the development of purpuric papules and resolving with ecchymoses and postinflammatory hyperpigmentation. Laboratory studies and skin biopsy results were consistent with UV.

  10. The impact of {sup 18}F-FDG PET on the management of patients with suspected large vessel vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Fuchs, Martin; Rasch, Helmut; Berg, Scott; Ng, Quinn K.T.; Mueller-Brand, Jan; Walter, Martin A. [University Hospital, Institute of Nuclear Medicine, Basel (Switzerland); Briel, Matthias [University Hospital Basel, Institute for Clinical Epidemiology and Biostatistics, Basel (Switzerland); McMaster University, Department of Clinical Epidemiology and Biostatistics, Hamilton, ON (Canada); Daikeler, Thomas; Tyndall, Alan [University Hospital Basel, Department of Rheumatology, Basel (Switzerland); Walker, Ulrich A. [Felix Platter Spital, Department of Rheumatology of Basle University, Basel (Switzerland); Raatz, Heike [University Hospital Basel, Institute for Clinical Epidemiology and Biostatistics, Basel (Switzerland); Jayne, David [Addenbrooke' s Hospital, Vasculitis and Lupus Unit, Cambridge (United Kingdom); Koetter, Ina [University Hospital Tuebingen, Department of Internal Medicine II, Tuebingen (Germany); Blockmans, Daniel [University Hospital Gasthuisberg, Department of General Internal Medicine, Leuven (Belgium); Cid, Maria C.; Prieto-Gonzalez, Sergio [Hospital Clinic, University of Barcelona, IDIBAPS, Department of Systemic Autoimmune Diseases, 08036-Barcelona (Spain); Lamprecht, Peter [University Hospital of Schleswig-Holstein, Department of Rheumatology, Luebeck (Germany); Salvarani, Carlo [Arcispedale S. Maria Nuova, Department of Rheumatology, Reggio Emilia (Italy); Karageorgaki, Zaharenia [Agios Dimitrios General Hospital, 1st Department of Internal Medicine, Thessaloniki (Greece); Watts, Richard [University of East Anglia, Norwich Medical School, Norwich (United Kingdom); Ipswich Hospital NHS Trust, Ipswich (United Kingdom); Luqmani, Raashid [Nuffield Orthopaedic Centre, Department of Rheumatology, Oxford (United Kingdom)

    2012-02-15

    We aimed to assess the impact of {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET) on the management of patients with suspected large vessel vasculitis. An international expert panel determined diagnoses and clinical management in patients with suspected large vessel vasculitis, with and without the results of {sup 18}F-FDG PET, respectively. The accuracy of the clinical diagnosis and the resulting clinical management with and without the {sup 18}F-FDG PET results were compared using logistic regression models. The analysis included 30 patients referred to a tertiary care centre with large vessel vasculitis and 31 controls. {sup 18}F-FDG PET had an overall sensitivity of 73.3% [95% confidence interval (CI) 54.1-87.7%], a specificity of 83.9% (95% CI 66.3-94.5%), a positive predictive value of 81.5% (95% CI 61.9-93.7%) and a negative predictive value of 76.5% (95% CI 58.8-89.3%). The diagnostic accuracy of {sup 18}F-FDG PET was higher in patients not receiving immunosuppressive drugs (93.3 vs 64.5%, p = 0.006). Taken in context with other available diagnostic modalities, the addition of {sup 18}F-FDG PET increased the clinical diagnostic accuracy from 54.1 to 70.5% (p = 0.04). The addition of {sup 18}F-FDG PET increased the number of indicated biopsies from 22 of 61 patients (36.1%) to 25 of 61 patients (41.0%) and changed the treatment recommendation in 8 of 30 patients (26.7%) not receiving immunosuppressive medication and in 7 of 31 patients (22.6%) receiving immunosuppressive medication. {sup 18}F-FDG PET is a sensitive and specific imaging tool for large vessel vasculitis, especially when performed in patients not receiving immunosuppressive drugs. It increases the overall diagnostic accuracy and has an impact on the clinical management in a significant proportion of patients. (orig.)

  11. The Role of 18F-FDG PET/CT in Large-Vessel Vasculitis: Appropriateness of Current Classification Criteria?

    Directory of Open Access Journals (Sweden)

    H. Balink

    2014-01-01

    Full Text Available Patients with clinical suspicion of large-vessel vasculitis (LVV may present with nonspecific signs and symptoms and increased inflammatory parameters and may remain without diagnosis after routine diagnostic procedures. Both the nonspecificity of the radiopharmaceutical 18F-FDG and the synergy of integrating functional and anatomical images with PET/CT offer substantial benefit in the diagnostic work-up of patients with clinical suspicion for LVV. A negative temporal artery biopsy, an ultrasonography without an arterial halo, or a MRI without aortic wall thickening or oedema do not exclude the presence of LVV and should therefore not exclude the use of 18F-FDG PET/CT when LVV is clinically suspected. This overview further discusses the notion that there is substantial underdiagnosis of LVV. Late diagnosis of LVV may lead to surgery or angioplasty in occlusive forms and is often accompanied by serious aortic complications and a fatal outcome. In contrast to the American College of Rheumatology 1990 criteria for vasculitis, based on late LVV effects like arterial stenosis and/or occlusion, 18F-FDG PET/CT sheds new light on the classification of giant cell arteritis (GCA and Takayasu arteritis (TA. The combination of these observations makes the role of 18F-FDG PET/CT in the assessment of patients suspected for having LVV promising.

  12. Vasculitis in Systemic Sclerosis

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    Lily Kao

    2010-01-01

    Full Text Available Systemic sclerosis (SSc is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical features of SSc with the renal manifestation of ANCA-associated glomerulonephritis. Other vasculitic syndromes, including large-vessel vasculitis, Behcet's disease, cryoglobulinemia, and polyarteritis nodosa, are rarely encountered in SSc patients. ANCA-associated vasculitis needs to be considered as a differential diagnosis in SSc patients presenting with renal insufficiency, as renal manifestations may result from distinct disease processes and require appropriate diagnostic testing and treatment.

  13. 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.

  14. Granulomatous vasculitis.

    Science.gov (United States)

    Marzano, A V; Balice, Y; Tavecchio, S; Desimine, C; Colombo, A; Berti, E

    2015-04-01

    Granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Wegener's granulomatosis and Churg-Strauss Syndrome respectively, are systemic granulomatous vasculitides affecting small- and medium-sized blood vessels. Both GPA and EGPA are included within the group of antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitides, on the basis of the detection of such autoantibodies in a significant proportion of affected patients. Two main settings of GPA, possibly overlapping each other, are recognized: a localized form, which is limited to the upper airways but is highly relapsing and refractory, and a diffuse form, which is initially more severe but then less commonly recurrent. In EGPA, a prodromic phase characterized by asthma and rhino-sinusitis is followed by an eosinophilic phase, marked by peripheral eosinophilia, and then by a vasculitic phase, in which skin lesions are a prominent feature together with peripheral neuropathy and renal involvement. Polymorphic cutaneous manifestations can occur during the course of both GPA and EGPA, and include palpable purpura, livedo reticularis, papules, nodules, vesiculo-bullae and necrotic-ulcerative lesions most commonly involving the lower extremities; pyoderma gangrenosum-like ulcers and lesions resembling erythema multiforme have been described in GPA and EGPA, respectively. Oral involvement is not uncommon in GPA and may manifest as nonspecific erosive lesions or as a hyperplastic gingivitis named strawberry gingivitis. Considering that skin involvement is common in ANCA-associated vasculitides and may also be their presenting sign, the role of dermatologist is crucial in the early diagnosis of these forms as well as of vasculitis in general.

  15. Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement.

    Science.gov (United States)

    Vassallo, C; Derlino, F; Croci, G A; Brazzelli, V; Borroni, G

    2015-02-01

    Granuloma faciale (GF) is a rare cutaneous condition of unknown origin, that usually presents as one or more brown-purple papules, plaques and/or nodules, localized mostly on the face, although extrafacial lesions can also occur. Eosinophilic angiocentric fibrosis (EAF) is regarded as the mucosal counterpart of GF. Histologically, it has been described as a persistent leukocytoclastic vasculitis, with a dense polymorphous inflammatory infiltrate in the superficial and mid dermis, typically sparing the subpapillary dermis, the so called grenz zone. The presence of eosinophils is considered a characteristic feature of the disease. All the cases of GF seen at the Dermatology Unit from 2002 to 2013 were considered and reviewed, both clinically and histopathologically. Only cases with consistent clinical findings of GF, and accurate patient's history were considered. Ten cases of GF were reviewed for both histological specificity and clinico-pathological correlation. Two patients presented extrafacial lesions. One patient had involvement of nasal mucosa. Two patients suffered from associated rheumatological diseases. The most frequent histopathologic features were the presence of a grenz zone and eosinophils in the infiltrate, but also adnexal involvement was often present; vascular changes were constant, yet leukocytoclastic vasculitis could be recorded only in four cases. Fibrosis or sclerosis were always absent. Clinical pictures of the patients treated demonstrated a complete remission of the lesions, without scarring. However, a complete enduring healing was observed only in two patients, and relapse or incomplete remission of the disease was the rule. In conclusion a review of clinicopathological findings of ten patients affected by GF was made and new details of the disease presented.

  16. The use of muscle biopsy in the diagnosis of systemic vasculitis affecting small to medium-sized vessels: a prospective evaluation in Japan.

    Science.gov (United States)

    Nunokawa, T; Yokogawa, N; Shimada, K; Enatsu, K; Sugii, S

    2016-01-01

    The aim of this study was to assess the use of muscle biopsy for histopathological confirmation of small vessel vasculitis (SVV) or medium vessel vasculitis (MVV). Muscle biopsies were performed for all consecutive cases of suspected SVV or MVV seen at Tokyo Metropolitan Tama Medical Centre between February 2012 and May 2014 except those for which a skin or renal biopsy was indicated. Forty-nine patients underwent muscle biopsies. All patients were followed for a minimum of 6 months. Diagnosis of SVV or MVV was made in 35 patients. An unrelated condition was diagnosed in 11 patients and no diagnoses were made in three patients. Of the 35 patients in whom SVV or MVV was diagnosed, positive muscle biopsies were obtained in 20 patients [15 microscopic polyangiitis (MPA), three polyarteritis nodosa (PAN), and two eosinophilic granulomatosis with polyangiitis (EGPA)], while other findings led to the same diagnosis in 15 (seven MPA, four GPA, three PAN, and one rheumatoid vasculitis). The sensitivity of the muscle biopsy was 57% [20/35; 95% confidence interval (CI) 50-57]. Of 13 patients presenting with peripheral neuropathy, the muscle biopsy demonstrated vasculitis in nine patients, with 75% sensitivity (9/12; 95% CI 69-75). There were no complications in the procedure apart from delayed wound healing in one patient. Muscle biopsy is a safe method that offers a high diagnostic yield for SVV or MVV, especially in patients with vasculitic neuropathy.

  17. High-Resolution Vessel Wall Magnetic Resonance Imaging in Varicella-Zoster Virus Vasculitis.

    Science.gov (United States)

    Tsivgoulis, Georgios; Lachanis, Stefanos; Magoufis, Georgios; Safouris, Apostolos; Kargiotis, Odysseas; Stamboulis, Elefterios

    2016-06-01

    Varicella-zoster virus vasculopathy is a rare but potentially treatable condition. Diagnosis has been based on angiography, brain magnetic resonance imaging (MRI), and cerebrospinal fluid analysis. High-resolution vessel wall MRI may aid to the diagnosis by differentiating inflammation from other vessel wall pathologies. We present the characteristic MRI findings of this condition in a young patient presenting with ischemic stroke. Copyright © 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  18. How Is Vasculitis Diagnosed?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  19. Living with Vasculitis

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  20. What is Vasculitis?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  1. How Is Vasculitis Treated?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  2. Types of Vasculitis

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  3. Renal involvement in hypocomplementaemic urticarial vasculitis syndrome: a report of three paediatric cases.

    Science.gov (United States)

    Pasini, Andrea; Bracaglia, Claudia; Aceti, Arianna; Vivarelli, Marina; Lavacchini, Alessandra; Miniaci, Angela; De Benedetti, Fabrizio; Montini, Giovanni

    2014-08-01

    To describe the diagnosis and management of renal disease in three paediatric cases of hypocomplementaemic urticarial vasculitis syndrome (HUVS). Three children who were diagnosed with HUVS and developed abnormalities of renal function during the disease course are described. Urinary findings were heterogeneous: all the patients developed persistent microhaematuria, which was isolated in patient 1, associated with mild proteinuria in patient 2 and with nephrotic syndrome in patient 3. Renal biopsies were performed in all the patients: patients 1 and 2, who had normal levels of serum autoantibodies, shared a full-house IF (C3, C1q and Ig deposits), compatible with an SLE-like disease; patient 3 showed negative staining for IgG and IgM, but developed positive anti-dsDNA without fulfilling criteria for the diagnosis of SLE. Renal involvement in HUVS is probably more frequent and more severe than in adults and may appear later. Isolated microhaematuria can be the only sign of subclinical renal involvement: its role should not be underestimated and a renal biopsy should be considered. Previous observations of rapidly progressive nephritis and consequent end-stage renal disease in children suggest the need for prompt diagnosis and treatment of renal involvement. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  4. Vestibular Involvement in Patients With Otitis Media With Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

    Science.gov (United States)

    Morita, Yuka; Takahashi, Kuniyuki; Izumi, Shuji; Kubota, Yamato; Ohshima, Shinsuke; Horii, Arata

    2017-01-01

    Otitis media (OM) with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) is a novel concept of ear disease that is characterized by progressive mixed or sensorineural hearing loss with occasional systemic involvement. Considering the accumulating knowledge about the characteristics of and treatment for auditory dysfunction in OMAAV, the objective of this study was to investigate the vestibular function and symptoms of patients with OMAAV. Retrospective chart review. University hospital. Thirty-one OMAAV patients met criteria proposed by the OMAAV study group in Japan. Clinical characteristics and vestibular tests. Eleven of 31 OMAAV patients had vestibular symptoms; 3 patients had acute vertigo attack with sudden hearing loss and 8 patients had chronic dizziness. Episodic vertigo was not seen in any of the patients. Three patients who received a less intensive therapy without immunosuppressive agents developed intractable persistent dizziness. All symptomatic patients and six of the nine OMAAV patients without vestibular symptoms showed unilateral or bilateral caloric weakness; therefore, vestibular involvement was present in 84% of OMAAV patients. Gain of vestibulo-ocular reflex was reduced in symptomatic patients. The eye-tracking test and optokinetic nystagmus revealed no evidence of central dysfunction. Vestibular dysfunction was seen in 84% of OMAAV patients. One-third of OMAAV patients showed vestibular symptoms such as acute vertigo attack or chronic dizziness, which are of peripheral origin. One-third of the symptomatic patients developed intractable dizziness. Initial intensive treatment by combination therapy with steroid and immunosuppressive agents may be essential for preventing the development of intractable dizziness.

  5. A Unique Case of Systemic Lupus Erythematosus Pelvic Vasculitis

    Directory of Open Access Journals (Sweden)

    Pamela Traisak

    2016-01-01

    Full Text Available The clinical presentation of Systemic Lupus Erythematosus (SLE is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA, polyarteritis nodosa (PAN, and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA, among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition.

  6. Urticarial Vasculitis-Associated Intestinal Ischemia

    National Research Council Canada - National Science Library

    Wong, Uni; Yfantis, Harris; Xie, Guofeng

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome...

  7. 3D-black-blood 3T-MRI for the diagnosis of thoracic large vessel vasculitis: A feasibility study

    Energy Technology Data Exchange (ETDEWEB)

    Treitl, Karla Maria; Saam, Tobias [Institute for Clinical Radiology, LMU Munich, Munich (Germany); German Center for Cardiovascular Disease Research (DZHK e.V.), Munich (Germany); Maurus, Stefan; Sommer, Nora Narvina; Coppenrath, Eva; Treitl, Marcus [Institute for Clinical Radiology, LMU Munich, Munich (Germany); Kooijman-Kurfuerst, Hendrik [Philips Healthcare, Hamburg (Germany); Czihal, Michael; Hoffmann, Ulrich [LMU Munich, Division of Vascular Medicine, Medical Clinic and Policlinic IV, Munich (Germany); Dechant, Claudia; Schulze-Koops, Hendrik [LMU Munich, Division of Rheumatology and Clinical Immunology, Medical Clinic and Policlinic IV, Munich (Germany)

    2017-05-15

    To evaluate the feasibility of T1w-3D black-blood turbo spin echo (TSE) sequence with variable flip angles for the diagnosis of thoracic large vessel vasculitis (LVV). Thirty-five patients with LVV, diagnosed according to the current standard of reference, and 35 controls were imaged at 3.0T using 1.2 x 1.3 x 2.0 mm{sup 3} fat-suppressed, T1w-3D, modified Volumetric Isotropic TSE Acquisition (mVISTA) pre- and post-contrast. Applying a navigator and peripheral pulse unit triggering (PPU), the total scan time was 10-12 min. Thoracic aorta and subclavian and pulmonary arteries were evaluated for image quality (IQ), flow artefact intensity, diagnostic confidence, concentric wall thickening and contrast enhancement (CWT, CCE) using a 4-point scale. IQ was good in all examinations (3.25 ± 0.72) and good to excellent in 342 of 408 evaluated segments (83.8 %), while 84.1 % showed no or minor flow artefacts. The interobserver reproducibility for the identification of CCE and CWT was 0.969 and 0.971 (p < 0.001) with an average diagnostic confidence of 3.47 ± 0.64. CCE and CWT were strongly correlated (Cohen's k = 0.87; P < 0.001) and significantly more frequent in the LVV-group (52.8 % vs. 1.0 %; 59.8 % vs. 2.4 %; P < 0.001). Navigated fat-suppressed T1w-3D black-blood MRI with PPU-triggering allows diagnosis of thoracic LVV. (orig.)

  8. Radiocontrast-Related Leukocytoclastic Vasculitis Misdiagnosed as Diabetic Foot Ulcer in a Type 2 Diabetic Patient: A Case Report

    Directory of Open Access Journals (Sweden)

    Barış Sarıakçalı

    2013-09-01

    Full Text Available The skin is the most affected tissue by many vasculitis syndromes. Leukocytoclastic vasculitis is the most common type of vasculitis syndrome and involves the small vessels. A long list of causative factors has been reported for leukocytoclastic vasculitis. Here, we present a type 2 diabetic patient who had purpuric skin lesions predominantly on the lower limbs and acute renal failure overriding to underlying chronic kidney disease due to leukocytoclastic vasculitis associated with radiocontrast administration. He was initially diagnosed as having diabetic foot ulcer at our outpatient clinic. After single dose betamethasone depot (9.6 mg i.m., skin eruptions paled and improved; renal function showed an improvement on the following days. There are few case reports on the relationship of radiocontrast agent with leucocytoclastic vasculitis. Our case had leukocytoclastic vasculitis due to radiocontrast agent exposure which is very rare in the literature. Turk Jem 2013; 17: 78-80

  9. Granulomatous slack skin - seven years follow-up of a case with features of "parapsoriasis en plaques", muscle involvement, granulomatous vasculitis, and necrobiotic changes.

    Science.gov (United States)

    ChiriŢă, Aurel Doru; Mărgăritescu, Irina

    2016-01-01

    Granulomatous slack skin (GSS) represents an extremely rare variant of mycosis fungoides with only 70 cases reported in the literature to date. It is characterized clinically by the occurrence of bulky, pendulous skinfolds, usually located in flexural areas and histologically by an infiltrate composed of small neoplastic T-lymphocytes joined by granulomatous inflammation with scattered multinucleated giant cells containing nuclei arranged in a wreath-like fashion. Since its first description, very rare cases of GSS with muscle involvement, large vessels involvement, or necrobiotic changes have been reported. We present an extraordinary case of GSS with all these unusual features developing in the lesions of the same patient. The long follow-up of seven years allowed us to document the evolution of each lesion. Some lesions appeared and evolved in a manner very reminiscent of those of "parapsoriasis en plaques", others were classical GSS lesions, and still others developed large ulcerated lesions. These ulcerated lesions consistently failed to respond to conventional wound therapy, skin directed therapy [retinoids + psoralen combined with ultraviolet A (PUVA)-therapy], and interferon-alpha therapy. Remarkably, the ulcers completely healed when systemic corticosteroids were added. We hence postulate that the ulcers appeared because of large vessel vasculitis rather than tumoral direct destruction.

  10. Levamisole-Induced Vasculitis: A Characteristic Cutaneous Vasculitis Associated With Levamisole-Adulterated Cocaine.

    Science.gov (United States)

    Roberts, Jordan A; Chévez-Barrios, Patricia

    2015-08-01

    Levamisole-induced vasculitis is a characteristic cutaneous vasculitis syndrome associated with the use of levamisole-adulterated cocaine. Patients will typically present with a painful, purpuric rash in a retiform or stellate pattern with or without central necrosis involving the extremities, trunk, nasal tip, digits, cheeks, and/or ears. A history of cocaine abuse can be elicited. Histologic features include microvascular thrombi and/or leukocytoclastic vasculitis involving small vessels of the superficial and deep dermis. Epidermal involvement is variably seen. Laboratory findings include leukopenia, neutropenia (including agranulocytosis), elevated erythrocyte sedimentation rate, normal coagulation studies, and positive autoantibodies including perinuclear and cytoplasmic antineutrophil cytoplasmic antibodies, antinuclear antibody, and lupus anticoagulant. Differential diagnosis includes other microscopic vasculitides, and clinical and laboratory correlation with histologic findings is essential. Lesions typically resolve with the cessation of cocaine use. Because of the treatment implications and rising incidence of this entity, rapid and accurate diagnosis is essential.

  11. In vasculitis of small muscular arteries, activation of vessel-infiltrating CD8 T cells seems to be antigen-independent.

    Science.gov (United States)

    Kobayashi, Mikiko; Ogawa, Eisaku; Okuyama, Ryuhei; Kanno, Hiroyuki

    2017-11-11

    The etiology of polyarteritis nodosa (PAN) and localized PAN is still unknown, although a T cell-mediated immune mechanism has been considered. CD8 T cells participate not only in the antigen-dependent adaptive immune system, but also in the antigen-independent innate immune system. Non-antigen-activated CD8 T cells express a unique phenotype: granzyme B (GrB) positive /CD25 negative /programmed death-1 (PD-1) negative. The aims of this study were to assess the participation of T cells, especially innate CD8 T cells, in the development of vasculitis. Twenty-eight consecutive cases of skin biopsy specimens with cutaneous vasculitis of small muscular arteries (CVSMA) were retrieved. The series comprises of 21 cases of cutaneous arteritis, three cases of PAN, and four cases of rheumatoid vasculitis. Cases of antineutrophil cytoplasmic antibody-associated vasculitis were excluded. The phenotypes of infiltrating lymphocytes in vasculitis lesions were evaluated by immunohistochemistry. In most cases of CVSMA, the number of CD8 T cells infiltrating the intima was higher than that of CD4 T cells, and significant numbers of GrB-positive cells, which represent activated CD8 T cells, were observed. However, GrB/CD25-double-positive cells, which correspond to antigen-activated T cells, were very few in a small number of cases. Cells positive for PD-1, which is also expressed on antigen-activated CD8 T cells, were not detected. We conclude that a T cell-mediated immune mechanism, involving cytotoxic CD8 T cells, may play a role in the development of CVSMA. Low expression of CD25 in activated CD8 T cells suggests that activation was antigen-independent.

  12. Active necrotizing cerebral vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Goel, Deepa; Reddy, S Rajashekhar; Sundaram, Challa; Prayaga, Aruna K; Rajasekhar, Liza; Narsimulu, Gumdal

    2007-12-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with varied clinical manifestations. Focal cortical brain infarcts and CNS infections are the most common neuropathological features reported in most studies. This report describes a 32-year-old woman who had repeated episodes of strokes over 5 years. In view of polyarthritis, oral ulcers, presence of high titres of serum antinuclear antibodies, high titres of double-stranded DNA and strokes, she was treated as SLE. Despite prolonged immunosuppressive therapy with azathioprine and pulse cyclophosphamide, she succumbed to a brainstem stroke. Complete body autopsy showed multiple cerebral cortical and brainstem infarcts with fibrinoid necrosis of the vessel wall. Renal infarction with healed vasculitis and systemic vasculitis involving small vessels was seen. Extensive thrombosis was remarkable by its absence. Active necrotizing vasculitis of cerebral and renal vessels is a rare complication of SLE, which contributed to a fatal outcome in this patient.

  13. Upper Respiratory Tract Symptoms, Renal Involvement and Vasculitis: A Case Report and Review of Wegener Granulomatosis

    Science.gov (United States)

    Said, Mohd Shahrir Mohamed

    2010-01-01

    Wegener’s Granulomatosis is a condition associated with systemic vasculitis which can present with upper respiratory tract symptoms initially. On September 2001, a 15-year-old girl presented with symptoms of nasal block for 3 weeks. She later developed joint pains and worsening renal status requiring dialysis. A renal biopsy was performed which showed pauci-immune cresentric glomerulonephritis. Her cANCA levels were positive. She was treated with oral cyclophosphamide and steroids and later responded. Keywords Wegener granulomatosis; Young girl; Cyclophophamide; cANCA PMID:21629538

  14. THROMBOTIC AND THROMBOEMBOLIC COMPLICATIONS IN SYSTEMIC VASCULITIS

    Directory of Open Access Journals (Sweden)

    E. V. Arseniev

    2017-01-01

    Full Text Available Nowadays, there is no doubt about the relationship between immune inflammation and the development of thrombosis due to the similarity of many pathogenetic machanisms underlying both pathological processes. Systemic vasculitis, along with other immuno- mediated inflammatory diseases, represent one of the most clear examples of such interaction. Thrombotic complications remain one of the most serious and life-threatening conditions that occur in patients with autoimmune diseases, in particular systemic vasculitis. This serves as powerful stimulus for studing the problem of hypercoagulation, which often accompanies the course of systemic vasculitis. Of interest are thrombosis that occure both in the injury of large vessel and in the involvement of medium and small vessels in the pathological process. According to the literature, thrombotic and thromboembolic complications are most common in ANCA-associated vasculitis and Behçet’s disease. This review discusses current studies regarding features of clinical picture and mechanisms of thrombosis development in systemic vasculitis. Because of these studies it became clear that for some vasculitis a high frequency of both arterial and venous thrombosis is characteristic. At the same time, other vasculitis are accompanied by high risk of only venous thrombosis. Finally, thrombosis and thromboembolism are quite rare complications for some vasculitis. Among the presented mechanisms of thrombosis involvement, disruptions of the hemostasis are widely considered. For examples, the presents the results of researches, which is actively discussed the role of neutrophil extracellular traps and antiendothelial cell antibodies in the development on thrombotic complictions. Special attention is paid to the possible role of some disorders of hemostasis, such as polymorphism V coagulation factor G1691FA and prothrombin G20210A. To search for literature sources, the following queries were used: “vasculitis”,

  15. Systemic panca-associated vasculitis with central nervous involvement causing recurrent myelitis: case report

    Directory of Open Access Journals (Sweden)

    Bull Matthew D

    2010-11-01

    Full Text Available Abstract Background We report on the case of an established perinuclear antineutrophil cytoplasmic antibody (pANCA associated renal vasculitis being treated with prednisolone and rituximab, where the patient presented with leg weakness, urinary and faecal incontinence and buttock pain consistent with transverse myelitis. Case presentation The patient underwent MRI scanning showing patchy cord enhancement from T10 to the conus, which was suggestive of a cord malignancy. Prior to a cord biopsy, he was treated with steroids and a repeat MRI showed resolution of the original lesion with a new similar lesion from C7 to T3. Conclusions He made a marked recovery after further treatment with high dose steroids and plasma exchange.

  16. Refractory rheumatoid vasculitis.

    Science.gov (United States)

    Kumar, Ashok; Goel, Anshul; Lapsiwala, Mehul; Singhal, Suman

    2016-11-01

    Systemic rheumatoid vasculitis (SRV) can develop in rheumatoid arthritis of long duration and high disease activity. It most commonly manifests as cutaneous vasculitis and mononeuritis multiplex. This can involve any organ of the body and carries very high mortality. We report a case of a young male who had rheumatoid arthritis for the past 15 years and became refractory to standard drugs and anti-TNF agents. He subsequently developed SRV, which started as mononeuritis multiplex. Disease progressed to result in gangrene of hands and feet despite receiving intravenous cyclophosphamide. Intravenous immunoglobulin and rituximab also could not provide any response. Prolonged ICU stay resulted in critical care neuromyopathy. Central nervous system vasculitis developed even after repeated infusions of intravenous immunoglobulins and at last he died of complications. In this case report, we have presented rare and chronic protracted presentation of rheumatoid vasculitis involving skin, nerves, brain and testis, which was refractory to the recommended therapies.

  17. The prognostic value of baseline {sup 18}F-FDG PET/CT in steroid-naive large-vessel vasculitis: introduction of volume-based parameters

    Energy Technology Data Exchange (ETDEWEB)

    Dellavedova, L. [Ospedale Civile di Legnano, PET/CT Center - Nuclear Medicine Department, Legnano (Italy); University of Milan, Department of Health Sciences, Milan (Italy); Carletto, M.; Maffioli, L.S. [Ospedale Civile di Legnano, PET/CT Center - Nuclear Medicine Department, Legnano (Italy); Faggioli, P.; Sciascera, A.; Mazzone, A. [Ospedale Civile di Legnano, Internal Medicine Department, Legnano (Italy); Del Sole, A. [University of Milan, Department of Health Sciences, Milan (Italy)

    2016-02-15

    The aim of this study was to analyse if the result of a baseline {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scan, in large-vessel vasculitis (LVV) patients, is able to predict the course of the disease, not only in terms of presence/absence of final complications but also in terms of favourable/complicated progress (response to steroid therapy, time to steroid suspension, relapses, etc.). A total of 46 consecutive patients, who underwent {sup 18}F-FDG PET/CT between May 2010 and March 2013 for fever of unknown origin (FUO) or suspected vasculitis (before starting corticosteroid therapy), were enrolled. The diagnosis of LVV was confirmed in 17 patients. Considering follow-up results, positive LVV patients were divided into two groups, one characterized by favourable (nine) and the other by complicated progress (eight), on the basis of presence/absence of vascular complications, presence/absence of at least another positive PET/CT during follow-up and impossibility to comply with the tapering schedule of the steroid due to biochemical/symptomatic relapse. Vessel uptake in subjects of the two groups was compared in terms of intensity and extension. To evaluate the extent of active disease, we introduced two volume-based parameters: ''volume of increased uptake'' (VIU) and ''total lesion glycolysis'' (TLG). The threshold used to calculate VIU on vessel walls was obtained by the ''vessel to liver'' ratio by means of receiver-operating characteristic analysis and was set at 0.92 x liver maximum standardized uptake value in each patient. Measures of tracer uptake intensity were significantly higher in patients with complicated progress compared to those with a favourable one (p < 0.05). Measures of disease extension were even more significant and TLG emerged as the best parameter to separate the two groups of patients (p = 0.01). This pilot study shows that, in LVV patients, the

  18. Mannose-binding lectin (MBL) codon 54 (rs1800450) polymorphism predisposes towards medium vessel vasculitis in patients with systemic lupus erythematosus.

    Science.gov (United States)

    Negi, Vir Singh; Devaraju, Panneer; Misra, Durga Prasanna; Jain, Vikramraj K; Usdadiya, Jignesh Babulal; Antony, Paul T; Gulati, Reena

    2017-04-01

    Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with multiple etiological factors. Mannose-binding lectin (MBL) plays a key role in innate immunity by activating antibody-independent lectin complement pathway, opsonisation, phagocytosis, and immune complex (IC) clearance. Genetic polymorphisms in the promoter and coding regions of MBL gene affect the circulatory levels and biological activity of MBL. Defects in MBL can lead to defective opsonisation and, hence, hamper clearance of apoptotic debris, the persistence of which can drive autoantibody formation in lupus. The exon1 variants at codon 52, 54, and 57 have been reported to augment the risk of SLE in different ethnic populations. Three hundred South Indian Tamil patients with SLE and 460 age-, sex-, and ethnicity-matched controls were genotyped for three polymorphisms at codon 52, 54, and 57 in exon1 of MBL gene by Taqman real-time PCR. The three polymorphisms in exon1 of MBL were observed not to confer risk of developing SLE. However, MBL codon 54 rs1800450 polymorphism was associated with the development of medium vessel vasculitis and gangrene (OR-2.29, CI 95% 1.08-4.83, p = 0.02), whereas, the ancestral allele G conferred protection (OR-0.44, CI 95% 0.21-0.93, p = 0.02). Genetic variants in the exon1 of MBL gene per se are not risk factors for SLE in South Indian Tamils. However, the association of codon 54 (rs1800450) with medium vessel vasculitis suggests that it may be a genetic modifier of clinical phenotype in SLE.

  19. Refractory vasculitis

    NARCIS (Netherlands)

    Rutgers, Bram; Kallenberg, Cees G. M.

    Refractory vasculitis occurs in 4-5% of patients with anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Differences between therapies used for refractory disease are mostly reflected in the percentages of complete and partial remissions, but also in the number of serious side

  20. Onychomadesis Following Cutaneous Vasculitis.

    Science.gov (United States)

    Damevska, Katerina; Gocev, Gorgi; Pollozahani, Nora; Nikolovska, Suzana; Neloska, Lence

    2017-04-01

    vessels (8); chemotherapy agents temporary inhibit the mitotic activity in nail matrix (10); the detection of Coxackie virus in the shedding nail particle, following hand, foot, and mouth disease, suggests that the viral replication itself may directly damage the nail matrix (11). However, as nail changes are not unique, it may be difficult to incriminate a single etiological agent. Our patient presented with an onset of Beau lines seven weeks after the initial CLCV lesions, which suggests that vasculitis might have acted as a trigger for NMA. As the fingers were not affected by CLCV, an indirect effect of vasculitis is more plausible. Leukocytoclastic vasculitis is a small-vessel inflammatory disease mediated by a deposition of immune complexes. Thus, the circulating immune complexes may be involved in the damage of nail bed microvasculature. Considering that the patient had been receiving enalapril and diclofenac, it is less likely that those drugs were involved in the pathogenesis of NMA. Enalapril was continued, and the nail changes were resolved while patient was still on enalapril. Furthermore, diclofenac is a widely prescribed drug and its association with NMA is yet to be described in literature. We described a patient who developed Beau lines and onychomadesis following cutaneous leukocytoclastic vasculitis. This clinical observation can expand the spectrum of possible causes of nail matrix arrest.

  1. Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Aydogan, Kenan; Karadogan, Serap Koran; Adim, Saduman Balaban; Tunali, Sukran

    2006-09-01

    Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8% of SLE, while 50% of HUVS patients are diagnosed with SLE. We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE. As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.

  2. Idiopathic hypocomplementemic urticarial vasculitis-linked neuropathy.

    Science.gov (United States)

    Filosto, Massimiliano; Cavallaro, Tiziana; Pasolini, Giorgio; Broglio, Laura; Tentorio, Marta; Cotelli, Mariasofia; Ferrari, Sergio; Padovani, Alessandro

    2009-09-15

    Hypocomplementemic urticarial vasculitis (HUV) is a rare form of cutaneous small-vessel vasculitis characterized by recurrent episodes of urticaria and painful, tender, burning or itchy skin lesions, often associated with extracutaneous involvement but usually with no significant peripheral nerve damage. We describe a patient with an HUV of undetermined cause that developed a progressive multifocal sensory neuropathy whose symptoms were temporarily relieved by intravenous immunoglobulin treatment. Sural nerve biopsy showed asymmetrical multifocal nerve fiber loss and axon degeneration in nerve fascicles, a picture suggestive of ischemic damage as a likely result of a vasculitic process. We point out that an axonal neuropathy may complicate idiopathic HUV and suggest looking for peripheral nerve involvement in HUV patients.

  3. [Primary childhood vasculitis new classification criteria

    DEFF Research Database (Denmark)

    Herlin, T.; Nielsen, Susan

    2008-01-01

    Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria for the c......Primary vasculitis is seen in both adults and children, but some of the diseases like Kawasaki disease occur primarily in children. The Chapel Hill Classification Criteria for primary vasculitis refers to the size of vessels but has not been validated in children. Recently, new criteria...

  4. Endothelial cells, fibroblasts and vasculitis

    OpenAIRE

    Buckley, Christopher D.; Rainger, G.Ed; Nash, Gerard B; Raza, Karim

    2005-01-01

    One of the most important questions in vasculitis research is not why inflammation of blood vessels occurs but why it persists, often in a site-specific manner. In this review we illustrate how stromal cells, such as fibroblasts and pericytes, might play an important role in regulating the site at which vasculitis occurs. Smooth muscle cells and fibroblasts directly influence the behaviour of overlying vascular cells, amplifying the response of the endothelium to proinflammatory agents such a...

  5. Hypertrophic cardiomyopathy secondary to hepatitis C virus-related vasculitis.

    Science.gov (United States)

    Cavalli, Giulio; Berti, Alvise; Fragasso, Gabriele; De Cobelli, Francesco

    2016-12-01

    : Almost invariably associated with chronic HCV infection, cryoglobulinemic vasculitis is a small-vessel vasculitis commonly affecting the skin, kidneys, and peripheral nervous system. Cardiac involvement, possibly due to cardiac microcirculation involvement, is an utterly rare and severe complication. We describe a case of hypertrophic cardiomyopathy secondary to cryoglobulinemic vasculitis. Evaluation with transthoracic cardiac ultrasound and cardiac MRI evidenced severe left ventricular hypertrophy and diffuse hypokinesia, a marked decrease in left ventricular ejection fraction, and a subtle late enhancement of inferior and lateral left ventricular walls. Upon clinical stabilization, the patient received treatment with anti-CD20 monoclonal antibody rituximab. Clinical and radiological follow-up with cardiac ultrasound and cardiac MRI documented a dramatic and sustained clinical improvement, with marked reduction of left ventricular hypertrophy, resolution of late enhancement, recovery of left ventricular contractility and function.

  6. Urticarial Vasculitis-Associated Intestinal Ischemia

    Directory of Open Access Journals (Sweden)

    Uni Wong

    2016-01-01

    Full Text Available Urticarial vasculitis (UV is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia.

  7. Urticarial Vasculitis-Associated Intestinal Ischemia

    Science.gov (United States)

    Wong, Uni; Yfantis, Harris; Xie, Guofeng

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia. PMID:27190661

  8. 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

  9. 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.

  10. Vasculitis Pregnancy Registry

    Science.gov (United States)

    2017-04-27

    Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); Churg-Strauss Syndrome (CSS); Granulomatosis With Polyangiitis (GPA); Wegener's Granulomatosis; IgA Vasculitis; Henoch-Schoenlein Purpura (HSP); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Takayasu Arteritis (TAK); Urticarial Vasculitis; Systemic Vasculitis

  11. Drug-induced leukocytoclastic vasculitis: tigecycline a rare cause

    Directory of Open Access Journals (Sweden)

    Kalpana Bhairavarasu

    2015-01-01

    Full Text Available Drug-induced leukocytoclastic vasculitis is an inflammation of blood vessels triggered by various drugs. It presents with a localized skin rash but may involve the internal organ systems, including the gastrointestinal tract, kidneys, lungs, central nervous system, and joints. The clinical recognition of drug-induced vasculitis is very important because continued use of the culprit drug can be organ or life threatening. The prognosis is excellent if the disease is limited to the skin and diagnosed promptly. The use of tigecycline has recently increased due to resistance patterns of bacteria, and it is important to recognize this potential adverse effect of this drug and to diagnose and treat the patient early to achieve a favorable outcome. To best of our knowledge, we report the first case of tigecycline-induced leukocytoclastic vasculitis.

  12. [A case of polymyalgia rheumatica where a complication of large-vessel vasculitis was diagnosed by FDG-PET].

    Science.gov (United States)

    Shibata, Tomohiko; Shibata, Toshiko; Ozaki, Shoichi; Ichikawa, Yoichi; Itoh, Gen

    2009-04-01

    The patient was a 74-year-old female presenting with abrupt onset of fever and proximal muscle pains. She had been diagnosed with polymyalgia rheumatica (PMR). On physical examination, there was no tenderness or dilatation of the temporal artery and ocular fundi were normal. 18F-FDG-PET revealed accumulation of FDG in the aorta as well as in the bilateral subclavian arteries, which strongly suggested inflammation of the large blood vessels. Magnetic resonance angiography disclosed stenosis of the bilateral subclavian arteries, which was consistent with angitis. This case was considered to have developed PMR at an old age with positive HLA DR4, and to have a complication large-vessel giant cell arteritis (LV-GCA). Administration of prednisolone at a dose of 20 mg/day promptly relieved the fever and the myalgia as well. It is difficult to diagnose GCA in PMR if no tenderness or dilatation of the temporal artery is present. FDG-PET is considered useful, not only for exploration of tumors, but also for evaluation of inflammation of large vessels.

  13. Cocaine-Induced Vasculitis

    Directory of Open Access Journals (Sweden)

    Mark Berman

    2016-10-01

    Full Text Available The use of cocaine continues to grow worldwide. One of the possible side-effects of cocaine is vasculitis. Two distinct vasculitic syndromes have been described due to cocaine. One is cocaine-induced midline destructive lesion, secondary to a direct vasoconstrictor effect of cocaine, inducing ischemic necrosis of the septal cartilage and perforation of the nasal septum, mimicking findings of granulomatosis with polyangiitis in the upper airways. The other is ANCA-associated vasculitis, attributed to the levamisole component that contaminates about 70% of the cocaine. This type of vasculitis may be myeloperoxidase (MPO and proteinase 3 (PR3 positive, and its main manifestations are typical cutaneous findings, arthralgia, otolaryngologic involvement, and agranulocytosis. A high degree of suspicion and awareness is needed in order properly to diagnose and treat these patients.

  14. Imaging of systemic vasculitis in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Soliman, Magdy [The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada); Cairo University, Department of Medical Imaging, Cairo (Egypt); Laxer, Ronald; Yeung, Rae [The Hospital for Sick Children, Department of Rheumatology, Toronto, ON (Canada); Manson, David; Doria, Andrea S. [The Hospital for Sick Children, Department of Medical Imaging, Toronto, ON (Canada)

    2015-08-15

    The term ''systemic vasculitis'' encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children. (orig.)

  15. Equine pastern vasculitis: a clinical and histopathological study.

    Science.gov (United States)

    Psalla, Dimitra; Rüfenacht, Silvia; Stoffel, Michael H; Chiers, Koen; Gaschen, Véronique; Doherr, Marcus G; Gerber, Vincent; Welle, Monika M

    2013-11-01

    Equine pastern vasculitis is clinically challenging and the underlying aetiopathogenesis is unclear. The aims of this retrospective study were to establish histopathological criteria for pastern vasculitis, to look for an underlying cause, to investigate whether the histopathological lesions are associated with a distinct clinical picture, to assess if and how the clinical picture varies, and to determine the treatment response. Skin biopsies and clinical data from 20 horses with a diagnosis of vasculitis of the distal extremities were investigated and histology was compared to biopsies from healthy horses. It was concluded that intramural inflammatory cells, leukocytoclasia with nuclear dust, thickening and oedema of the vessel walls, and microhaemorrhages are highly specific histological findings in equine pastern vasculitis. Based on the feedback from the clinicians, the lesions were mostly seen on the lateral and medial aspects of un-pigmented legs. Lesions in white skin were characterised by exudation and crusts, whereas those in pigmented skin were alopecic and characterised by scaling. The response to treatment was poor and the prognosis guarded. No association was found between any of the histopathological findings and a distinct clinical picture. An underlying cause of equine pastern vasculitis could not be identified. Considering the large number of confounding factors, the causative agents are difficult to identify, but may involve drugs or a hypersensitivity reactions to yet unknown antigens. Copyright © 2013 Elsevier Ltd. All rights reserved.

  16. Clinical and immunological features of retinal vasculitis in systemic diseases

    Directory of Open Access Journals (Sweden)

    Paović Jelena

    2009-01-01

    Full Text Available Background/Aim. Vasculitis is a clinical, pathologic process characterized by inflammation and necrosis of blood vessel occurring anywhere in the body. The aim of the study was to present some clinical and immunologic features of retinal vasculitis in systemic diseases: systemic lupus erythematosus, multiple sclerosis, sarcoidosis, Behcet's diseases, and others. Methods. A total of 1 254 patients with uveitis were included in the study. The immunochemical diagnostic methods were used to determine the pathogenesis of ocular manifestations. Ocular manifestations were examined using biomicroscope, direct or indirect ophtalmoscopy. Results. Primary retinal vasculitis was diagnosed in 85/1254 (6.8% of total uveitis. In more than half of the cases of vasculitis (58.8%, both arteries and veins were involved in inflammatory process. Periphlebitis was diagnosed far more commonly (36.5% than periarteritis (4.7%. Retinal vasculitis associated with systemic lupus erythematosus was characterized by microinfarctions and the consequent foci of inflammatory cells or diseases of large arteries manifesting in vasospasm and occlusions. Cotton wool spots occurred in 38.3% and retinal hemorrhages in 34% of the cases. In this study periphlebitis of the retina was one of the less frequent ocular manifestations of multiple sclerosis. Comparing with the other changes of the retinal blood vessels, venous sheating occurred in 25.1% and occlusion and vein trombosis in 43.75% of the cases. Retinal vasculitis associated with chronic sarcoidosis occured in 37.5% of patients with sarcoidosis. The most frequent manifestation of ocular sarcoidosis was intermediary uveitis (43.75%. Anterior granulomatous uveitis occured in 37.5% of patients with sarcoidoses. Immune complexes occurred in 13/20 (65% of the patients. Antiretinal anti-S antibody in the serum occurred in 73% of the patients with retinal detachment as a complication of primary disease and in 25% those with vasculitis

  17. Clinical and molecular studies in ANCA associated vasculitis

    OpenAIRE

    Wendt, Mårten

    2014-01-01

    ANCA associated vasculitis (AAV) is a heterogeneous group of diseases characterised by sterile pauci-immune systemic small vessel inflammation and closely associated with the presence of anti-neutrophil cytoplasmatic antibodies (ANCA). Although AAV can affect any organ, the kidney, skin, lungs and upper and lower airways are most commonly involved. In some patients there is granuloma formation and in some asthma and eosinophilia, and based on this patients can be further classi...

  18. The role of biological agents in the management of large vessel vasculitis (LVV: a systematic review and meta-analysis.

    Directory of Open Access Journals (Sweden)

    Mohammed Osman

    Full Text Available BACKGROUND: Giant cell arteritis (GCA and Takayasu's arteritis (TAA are large vessel vasculitides (LVV for which corticosteroids (CS are the mainstay for treatment. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness. OBJECTIVE: To systematically review the effectiveness and safety of biological agents in patients with LVV. METHODS: We searched 5 electronic databases (inception to October 2012 and conference abstracts with no language restrictions. Two reviewers independently selected studies, extracted data and assessed methodological quality. Our protocol was registered in PROSPERO. RESULTS: We included 25 studies (3 RCTs and 22 case series with ≥2 cases. 95 GCA and 98 TAA patients received biological agents. The RCTs using anti-TNF agents (infliximab, etanercept and adalimumab did not suggest a benefit in GCA. GCA patients receiving tocilizumab, in case series, achieved remission (19 patients and reduction of corticosteroid dose (mean difference, -16.55 mg/day (95% CI: -26.24, -6.86. In case series, 75 patients with refractory TAA treated with infliximab discontinued CS 32% of the time. Remission was variably defined and the studies were clinically heterogeneous which precluded further analysis. CONCLUSION: This systematic review demonstrated a weak evidence base on which to assess the effectiveness of biological treatment in LVV. Evidence from RCTs suggests that anti-TNF agents are not effective for remission or reduction of CS use. Tocilizumab and infliximab may be effective in the management of LVV and refractory TAA, respectively, although the evidence comes from case series. Future analytical studies are needed to confirm these findings.

  19. Ongoing Cerebral Vasculitis During Treatment of Rocky Mountain Spotted Fever.

    Science.gov (United States)

    Sun, Lisa R; Huisman, Thierry A G M; Yeshokumar, Anusha K; Johnston, Michael V

    2015-11-01

    Rocky Mountain spotted fever is a tickborne infection that produces a systemic small-vessel vasculitis; its prognosis is excellent if appropriate treatment is initiated early. Because the advent of effective antirickettsial therapies predates the widespread use of brain magnetic resonance imaging, there are limited data on the effect of untreated Rocky Mountain spotted fever infection on neuroimaging studies. We describe a 7-year-old girl with delayed treatment of Rocky Mountain spotted fever who suffered severe neurological impairment. Serial brain magnetic resonance images revealed a progressive "starry sky appearance," which is proposed to result from the same small vessel vasculitis that causes the characteristic skin rash of this infection. Neurological injury can continue to occur despite specific antirickettsial therapy in Rocky Mountain spotted fever. This child's clinical features raise questions about the optimal management of this infection, particularly the utility of immune modulating therapies in cases of delayed treatment and neurological involvement. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Pediatric Vasculitis Initiative

    Science.gov (United States)

    2017-05-01

    Wegeners Granulomatosis (Granulomatosis With Polyangiitis); Microscopic Polyangiitis; Churg Strauss Syndrome (Eosinophilic Granulomatosis With Polyangiitis); Polyarteritis Nodosa; Takayasu Arteritis; Primary CNS Vasculitis; Unclassified Vasculitis

  1. Hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Jara, Luis J; Navarro, Carmen; Medina, Gabriela; Vera-Lastra, Olga; Saavedra, Miguel A

    2009-12-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an uncommon immune complex-mediated entity characterized by urticaria with persistent acquired hypocomplementemia. First described in 1973, HUVS is associated with several systemic findings including leukocytoclastic vasculitis, severe angioedema, laryngeal edema, pulmonary involvement, arthritis, arthralgia, glomerulonephritis, and uveitis. These manifestations should be present for at least 6 months. Laboratory findings include low complement levels of classical pathway, namely C1q, C2, C3, and C4. The disease marker is the serum presence of anti-C1q antibodies. Treatment, based on disease severity, involves corticosteroids and other immunosuppressive agents that have demonstrated some success. Patients may have significant morbidity and mortality, most commonly caused by chronic obstructive pulmonary disease and acute laryngeal edema.

  2. Anti-neutrophil cytoplasmic antibody (ANCA) associated small-vessel vasculitis in a patient with diabetic nephropathy and autoimmune polyendocrinopathy syndrome (APS) Type 2: a case report.

    Science.gov (United States)

    Murray, Jonathan S; Baines, Laura A; Pearce, Simon H S; Ball, Steve; Leech, Nicola; Wood, Katrina M; Kanagasundaram, Nigel S

    2013-09-01

    We present a 42-year-old woman with pre-existing autoimmune polyendocrinopathy syndrome (APS) Type 2 and chronic kidney disease due to Type 1 diabetic nephropathy, who developed a rapid deterioration in renal function due to perinuclear anti-neutrophil cytoplasmic antibody (pANCA)-associated vasculitis. Although possibly a chance occurrence, ANCA have been detected more frequently in patients with a history of certain autoimmune diseases. Such an association may simply reflect an underlying tendency to immune system dysfunction in these patients and the finding of positive ANCA serology does not reliably herald the development of ANCA-associated vasculitis. However, our case illustrates that positive ANCA serology in such circumstances is not always a benign phenomenon and should still be interpreted within the clinical context. Moreover, clinicians managing patients with pre-existing autoimmune disease should maintain a low threshold for appropriate assessment should such patients develop evidence suggestive of vasculitis.

  3. Impact of Vasculitis on Employment and Income

    Science.gov (United States)

    2016-01-26

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatosis; Temporal Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa (PAN); Takayasu's Arteritis; Urticarial Vasculitis

  4. Demographic characteristics, aetiology, and assessment of treatment options in leukocytoclastic vasculitis

    Directory of Open Access Journals (Sweden)

    Alkim Unal Cakiter

    2017-04-01

    Full Text Available Introduction : Vasculitides are a heterogeneous group of diseases characterized by inflammation of the blood vessel walls. Etiological factors include infections, drugs, connective tissue diseases, and malignancies. Aim : To examine the demographic characteristics, etiological factors, and treatment options in 75 patients with leukocytoclastic vasculitis. Material and methods : The study included 75 patients diagnosed with leukocytoclastic vasculitis at our clinic. The patients’ medical records were reviewed to determine their age, sex, presence of systemic symptoms, possible etiological factors, laboratory results, types of cutaneous lesions, locations of the lesions, treatment options, and disease course. Results : There were 43 women and 32 men. Cutaneous lesions affected only the lower limbs in 60 of the 75 patients (80% and usually presented as palpable purpura (64%, n = 48. Arthralgia (26.7%, n = 20 was the most frequent extracutaneous symptom. Of the patients with secondary vasculitis, the most common causes were infections and drugs. The mean age of the patients with Henoch-Schönlein purpura was 26.8 years. There was no significant association between age and renal, gastrointestinal, or joint involvement. Conclusions : The most common form of vasculitis in our study was cutaneous leukocytoclastic vasculitis. In most of the patients it appeared to be idiopathic. Among drugs, antibiotics were the most common etiological factor. In 4 patients, the cutaneous leukocytoclastic vasculitis behaved like the paraneoplastic syndrome.

  5. Urticarial Vasculitis-Associated Intestinal Ischemia

    OpenAIRE

    Uni Wong; Harris Yfantis; Guofeng Xie

    2016-01-01

    Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a ca...

  6. Processing and analysis techniques involving in-vessel material generation

    Science.gov (United States)

    Schabron, John F [Laramie, WY; Rovani, Jr., Joseph F.

    2011-01-25

    In at least one embodiment, the inventive technology relates to in-vessel generation of a material from a solution of interest as part of a processing and/or analysis operation. Preferred embodiments of the in-vessel material generation (e.g., in-vessel solid material generation) include precipitation; in certain embodiments, analysis and/or processing of the solution of interest may include dissolution of the material, perhaps as part of a successive dissolution protocol using solvents of increasing ability to dissolve. Applications include, but are by no means limited to estimation of a coking onset and solution (e.g., oil) fractionating.

  7. Urticarial vasculitis and urticarial autoinflammatory syndromes.

    Science.gov (United States)

    Marzano, A V; Tavecchio, S; Venturini, M; Sala, R; Calzavara-Pinton, P; Gattorno, M

    2015-02-01

    Urticaria is a frequent disorder classified as acute and chronic forms, which presents with wheals that can be associated with angioedema. Several entities may manifest with urticarial skin lesions, encompassing a heterogeneous group of conditions that have to be differentiated from ordinary urticaria. This review is focused on two of these urticarial syndromes: urticarial vasculitis (UV), which represents the most important differential diagnosis with common urticaria, and autoinflammatory diseases such as cryopyrin-associated periodic syndromes (CAPS) and Schnitzler's Syndrome, both rare multisystem forms that may masquerade as common urticaria. UV is a small-vessel vasculitis with predominant skin involvement, characterized by wheals persisting for more than 24 hours, burning rather than itching and resolving with hyperpigmentation as well as by other cutaneous manifestations including purpura, papules, vesicles, bullae and necrotic-ulcerative lesions. Histology shows a classic pattern of leukocytoclastic vasculitis, with possible presence of upper dermal edema. CAPS are classified as three distinct entities: familial cold autoinflammatory syndrome, Muckle-Wells Syndrome and chronic infantile neurological cutaneous and articular syndrome, which represent a spectrum of disorders caused by different mutations in a single gene, NLRP3 (NOD-like receptor 3). This gene encodes for cryopyrin, an inflammasome protein that activates interleukin-1β, leading to an overproduction of this pivotal proinflammatory cytokine. Histologically, urticarial lesions are generally characterized by a perivascular neutrophilic infiltrate. Unlike urticaria, neither UV nor urticarial autoinflammatory syndromes do respond to antihistamines: thus, it is important not to misdiagnose such conditions in order to give the patients specific treatments, potentially preventing serious systemic complications.

  8. Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

    Science.gov (United States)

    2018-01-18

    Cryoglobulinemic Vasculitis (CV); Drug-induced Vasculitis; Eosinophilic Granulomatosis With Polyangiitis (EGPA); IgA Vasculitis; Isolated Cutaneous Vasculitis; Granulomatosis With Polyangiitis (GPA); Microscopic Polyangiitis (MPA); Polyarteritis Nodosa (PAN); Urticarial Vasculitis; Vasculitis

  9. Urticarial vasculitis in infancy

    Directory of Open Access Journals (Sweden)

    Kaur S

    2003-05-01

    Full Text Available Urticarial vasculitis is an uncommon manifestation of cutaneous vasculitis closely resembling chronic urticaria. It is an immune complex deposition disorder, which is not commonly observed in children. We report an 9-month-old infant with urticarial vasculitis and discuss its clinical course and differentiation from common urticaria.

  10. Vasculite cutânea de pequenos vasos: etiologia, patogênese, classificação e critérios diagnósticos - Parte I Small vessel cutaneous vasculitis: etiology, pathogenesis, classification and diagnostic criteria - Part I

    Directory of Open Access Journals (Sweden)

    Hebert Roberto Clivati Brandt

    2007-10-01

    Full Text Available Vasculite é a inflamação da parede dos vasos. Pode variar em gravidade desde doença autolimitada de um único órgão até doença grave com risco de morte por falência de múltiplos órgãos. Existem várias causas, embora só se apresente por poucos padrões histológicos de inflamação vascular. Vasos de qualquer tipo e em qualquer órgão podem ser afetados, resultando em ampla variedade de sinais e sintomas. Diferentes vasculites com apresentações clínicas indistinguíveis têm evolução e tratamento muito diferentes. Essa condição representa desafio para o médico, incluindo classificação, diagnóstico, exames laboratoriais pertinentes, tratamento e seguimento adequado. Neste artigo são revistos a classificação, a etiologia, a patogênese e os critérios diagnósticos das vasculites cutâneas.Vasculitis is an inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple-organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. This article reviews the classification, etiology, pathology and diagnostic criteria of cutaneous vasculitis.

  11. Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis.

    Science.gov (United States)

    Lee, Joyce Siong See; Loh, Teck Hiong; Seow, Swee Chong; Tan, Suat Hoon

    2007-06-01

    Biopsy specimens of lesions with clinical features of urticarial vasculitis often show a predominantly lymphocytic infiltrate with eosinophils and red blood cell extravasation. Only occasionally is a leukocytoclastic vasculitis encountered, confirming a diagnosis of urticarial vasculitis. The aim of this study was to assess the clinical presentation and histologic features of patients who meet the clinical criteria for urticarial vasculitis. Patients were recruited who had persistent urticarial lesions individually lasting longer than 24 hours, associated with at least 2 of 3 of the following: pain or tenderness; purpura or dusky changes; and resolution with hyperpigmentation. Patients were interviewed based on a standard questionnaire with regard to their symptoms. Blood tests and chest radiographs were performed to exclude systemic involvement and hypocomplementemia. Skin biopsy specimens were sent for histology and direct immunofluorescence. Of 22 patients recruited, 19 (86.4%) showed a predominantly lymphocytic infiltrate on histology. Three cases (13.6%) had a neutrophil-predominant infiltrate associated with a leukocytoclastic vasculitis. Twenty (90.9%) had a superficial perivascular infiltrate, and two (9.1%) had a superficial and deep perivascular infiltrate. In all, 21 biopsy specimens (95.5%) showed inflammatory cells within dermal blood vessel walls, obscuring the vessel outline in some. Endothelial cell swelling was seen in 20 biopsy specimens (90.9%), erythrocyte extravasation in 17 (77.3%), nuclear dust in 5 (22.7%), and fibrin extravasation in 2 (9.1%). Multivariate analysis revealed the following features to be independently associated with neutrophil predominance: fulfillment of all 3 minor criteria for urticarial vasculitis-like lesions (P = .007); presence of fibrin on histology (P urticarial vasculitis reveal that only a minority of patients has leukocytoclastic vasculitis. The majority has a lymphocyte-predominant histology, associated with

  12. Complement system activation in ANCA vasculitis : A translational success story?

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.; Heeringa, Peter

    2015-01-01

    The ANCA-associated vasculitides (AAV) are characterized by pauci-immune necrotizing small to medium size vessel vasculitis frequently including necrotizing crescentric glomerulonephritis. Neutrophil activation by ANCA appears a primary pathogenic event. More recently, the complement system has been

  13. What Are the Signs and Symptoms of Vasculitis?

    Science.gov (United States)

    ... more likely to occur in people of Scandinavian origin, but it can affect people of any race. ... of polyarteritis nodosa seem to be linked to hepatitis B or C infections. Mostly Small Vessel Vasculitis ...

  14. [Large vessel vasculitides].

    Science.gov (United States)

    Morović-Vergles, Jadranka; Puksić, Silva; Gracanin, Ana Gudelj

    2013-01-01

    Large vessel vasculitis includes Giant cell arteritis and Takayasu arteritis. Giant cell arteritis is the most common form of vasculitis affect patients aged 50 years or over. The diagnosis should be considered in older patients who present with new onset of headache, visual disturbance, polymyalgia rheumatica and/or fever unknown cause. Glucocorticoides remain the cornerstone of therapy. Takayasu arteritis is a chronic panarteritis of the aorta ant its major branches presenting commonly in young ages. Although all large arteries can be affected, the aorta, subclavian and carotid arteries are most commonly involved. The most common symptoms included upper extremity claudication, hypertension, pain over the carotid arteries (carotidynia), dizziness and visual disturbances. Early diagnosis and treatment has improved the outcome in patients with TA.

  15. Endothelial cells, fibroblasts and vasculitis.

    Science.gov (United States)

    Buckley, Christopher D; Rainger, G Ed; Nash, Gerard B; Raza, Karim

    2005-07-01

    One of the most important questions in vasculitis research is not why inflammation of blood vessels occurs but why it persists, often in a site-specific manner. In this review we illustrate how stromal cells, such as fibroblasts and pericytes, might play an important role in regulating the site at which vasculitis occurs. Smooth muscle cells and fibroblasts directly influence the behaviour of overlying vascular cells, amplifying the response of the endothelium to proinflammatory agents such as TNF-alpha and allowing enhanced and inappropriate leucocyte recruitment. An abnormal local vascular stromal environment can therefore influence local endothelial function and drive the persistence of local vascular inflammation. However, such local vascular inflammation can have distant effects on the systemic vascular system, leading to widespread endothelial cell dysfunction. Vascular endothelial dysfunction is common in a range of immune-mediated inflammatory diseases, is seen in multiple vascular beds, and is reversible following the induction of disease remission. The mechanisms that drive such systemic vascular endothelial dysfunction are unclear but factors such as TNF-alpha and CRP may play a role. Persistence of such widespread endothelial dysfunction in systemic vasculitis appears to have long-term consequences, leading to the acceleration of atherosclerosis and premature ischaemic heart disease. It may also underlie the accelerated atherosclerosis seen in other immune-mediated rheumatic diseases, such as rheumatoid arthritis.

  16. Neuroimaging of cerebral vasculitis; Bildgebung zerebraler Vaskulitiden

    Energy Technology Data Exchange (ETDEWEB)

    Wengenroth, M. [Universitaetsklinikum Schleswig-Holstein, Campus Luebeck, Institut fuer Neuroradiologie, Luebeck (Germany); Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer klinische Radiologie, Muenchen (Germany); Saam, T. [Klinikum der Ludwig-Maximilians-Universitaet, Institut fuer klinische Radiologie, Muenchen (Germany); Haehnel, S. [Universitaetsklinikum Heidelberg, Abteilung Neuroradiologie, Heidelberg (Germany)

    2016-01-15

    Cerebral vasculitis can have a variety of origins. Furthermore, there are no vasculitis-specific symptoms or imaging signs and vasculitis of the CNS can mimic many other neurological diseases, which require different treatment approaches. Thus, the clinical and radiological diagnosis of cerebral vasculitis is challenging. Magnetic resonance imaging (MRI) and MR angiography (MRA) should be the radiological imaging methods of choice to assess the degree of parenchymal damage and to detect vessel wall changes. If the results are unclear digital subtraction angiography (DSA) should be pursued in order to also detect changes in medium sized vessels. Vasculitis of small vessels cannot be detected by vascular imaging and requires brain or leptomeningeal biopsy. In this review we present the current diagnostic approach and a variety of imaging findings in cerebral vasculitis and discuss the main radiological differential diagnoses. (orig.) [German] Die zerebrale Vaskulitis kann viele Ursachen haben. Daher ist die klinische und radiologische Diagnose schwierig, auch weil es etliche vaskulitistypische Symptome und MR-Befunde bei anderen Erkrankungen gibt. Die ZNS-Vaskulitis kann zahlreiche andere neurologische Erkrankungen imitieren, die unterschiedlicher Therapie beduerfen. MRT und MR-Angiographie (MRA) sind die Verfahren der Wahl, mit denen die radiologische Diagnostik begonnen werden sollte, um den Parenchymschaden und den Gefaessbefall zu beurteilen. Bei unklaren Befunden kann eine digitale Subtraktionsangiographie (DSA) ergaenzt werden, um auch mittelgrosse Gefaesse beurteilen zu koennen. Der Befall kleiner Gefaesse kann nur bioptisch nachgewiesen werden. In der folgenden Uebersicht stellen wir das diagnostische Herangehen bei zerebraler Vaskulitis vor, praesentieren zahlreiche Befunde und diskutieren die wichtigsten radiologischen Differenzialdiagnosen. (orig.)

  17. Omalizumab for Urticarial Vasculitis

    DEFF Research Database (Denmark)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE) with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated...

  18. Hypocomplementemic urticarial vasculitis syndrome: an interdisciplinary challenge.

    Science.gov (United States)

    Grotz, Wolfgang; Baba, Hideo A; Becker, Jan U; Baumgärtel, Martin W

    2009-11-01

    Chronic urticaria often points the way to the diagnosis of a systemic disease, particularly when urticarial vasculitis can be demonstrated. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is considered to be an independent immunological disease. Selective literature review and consideration of the author's own clinical experience. The main manifestation of HUVS is chronic urticarial vasculitis with complement deficiency and the demonstration of C1q antibody in the serum. Multiple other organs are involved, sometimes severely. The diagnosis is confirmed by skin biopsy, which reveals leukocytoclastic vasculitis as a pathogenetic correlate of this systemic disease. Although HUVS is relatively rare, the medical specialists that might encounter it-ophthalmologists, rheumatologists, nephrologists, dermatologists, general practitioners, and pediatricians-should include it in their differential diagnoses whenever appropriate. Awareness of HUVS and rational diagnostic evaluation will lessen the chance of it being misdiagnosed as another type of systemic immunological disease and will reduce superfluous diagnostic testing in patients suffering from it.

  19. Systemic vasculitis in the geriatric population

    Directory of Open Access Journals (Sweden)

    Ari Stiel Radu Halpern

    2008-03-01

    Full Text Available Systemic vasculitis is a group of diseases characterized byinfl ammation of blood vessels with fi brinoid necrosis and resultingischemic changes. In the geriatric population it has been frequentlydescribed with patient age playing an important role in both thediagnosis and treatment of these diseases. Vasculitis may mimicmany common clinical situations of the elderly. They can affectmultiple organs at the same time with devastating consequences andchallenging diagnosis. The most common form of systemic vasculitisin older adults is giant cell arteritis or temporal arteritis that occursexclusively in people older than 50 years. Wegener granulomatosisand microscopic polyangiitis are also frequent in the geriatricpopulation. Treatment of vasculitis has developed to achieve a lowmortality and morbidity rate. Nevertheless, traditional therapeuticregimes are still related with severe side effects. New therapeuticroutines are being developed to induce a rapid remission allowingchange to a less iatrogenic treatment for remission maintenance.

  20. Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis.

    Science.gov (United States)

    Mizuno, Kana; Nguyen, Chuyen Thi Hong; Ueda-Hayakawa, Ikuko; Okamoto, Hiroyuki

    2017-05-01

    Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62-year-old female was diagnosed with sarcoidosis by chest-abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non-caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. Intracranial Aneurysms Involving Circle of Willis in a Child with Human Immunodeficiency Virus Associated Vasculitis- A Rare Case Report.

    Science.gov (United States)

    Lahoti, Amol Madanlal; Taori, Abhijit Kishor; Dhok, Avinash Parashuram; Rawat, Jitesh Subhash; Chandak, Nihar Umakant

    2017-07-01

    Intracranial Arterial Aneurysms (IAAs) are relatively rare in paediatric population and they account for at least 10%-15% of haemorrhagic strokes which occur during the first two decades of life. Here we present a unique and unusual case of Human Immunodeficiency Virus (HIV) infected child who presented with intracranial aneurysms with formation of collaterals and vasculopathy, demonstrating low viral count despite receiving adequate antiretroviral treatment. Intracranial vascular involvement, their complications and its incidence in these patients may become increasingly common as the management of human immunodeficiency virus infection continues to improve and afflicted patients survive for longer periods because of advancement in the antiretroviral treatment. Diagnosing aneurysm of cerebral circulation needs high degree of suspicion and correlation between clinical and radiological findings.

  2. Journey of Patients With Vasculitis From First Symptom to Diagnosis

    Science.gov (United States)

    2018-01-18

    Vasculitis; Systemic Vasculitis; Behcet's Disease; CNS Vasculitis; Cryoglobulinemic Vasculitis; Eosinophilic Granulomatous Vasculitis; Temporal Arteritis; Giant Cell Arteritis; Granulomatosis With Polyangiitis; Wegener Granulomatosis; Henoch Schonlein Purpura; IgA Vasculitis; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu Arteritis; Urticarial Vasculitis

  3. Urticaria and urticarial vasculitis.

    Science.gov (United States)

    Guha, Bhuvana; Youngberg, George; Krishnaswamy, Guha

    2003-01-01

    Urticaria and urticarial vasculitis may be triggered by allergens, infection, autoimmunity and other immunological conditions. Careful evaluation, skin biopsy and specific laboratory tests can assist in diagnosis. The appropriate use of antihistamines, glucocorticoids, and other immunomodulators are discussed.

  4. The role of first-pass perfusion deficit in the detection of cardiac subendocardial manifestation in patients with autoimmune vasculitis.

    Science.gov (United States)

    Pfeil, Alexander; Lehmann, Gabriele; Böttcher, Joachim; Wolf, Gunter; Hansch, Andreas

    2013-01-01

    This report describes the potential of cardiac magnetic resonance imaging (cMRI) based on myocardial first-pass perfusion imaging in the visualization of cardiac manifestations in autoimmune vasculitis, which in the heart are typically localized at the level of small subendocardial vessels. Two patients with primary or secondary autoimmune vasculitis were investigated in this study. Myocardial first-pass perfusion imaging was performed using an ECG-gated T1-weighted MRI sequence after the injection of intravenous bolus of gadolinium chelate. In both cases, the cMRI showed findings of subendocardial first-pass perfusion deficit (FPPD), a phenomenon so far described as microvascular obstruction (MVO) only in patients with acute cardiac infarction due to thromboembolic obstruction of small myocardial vessels. The two patients showed local subendocardial and myocardial hypoenhancement (characterized by a darker appearance than normal myocardial tissue), which is the typical morphological stigma of FPPD initially after injection of contrast media. The perfusion deficit, although morphologically very similar to the well-known phenomenon of MVO in acute cardiac infarction, was conceivably caused by different vasculitis-specific mechanisms such as occlusion of the microvasculature with erythrocytes, neutrophils and cellular debris. This study indicates that FPPD is useful for the non-invasive assessment of the microvasculature in patients with acute cardiac involvement in primary and secondary vasculitis.

  5. Intravenous immunoglobulin therapy for hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus in a child.

    Science.gov (United States)

    Yamazaki-Nakashimada, Marco A; Duran-McKinster, Carola; Ramírez-Vargas, Nadia; Hernandez-Bautista, Victor

    2009-01-01

    Hypocomplementemic urticarial vasculitis is a type of urticarial vasculitis with multisystemic involvement and poor prognosis, sometimes associated with systemic lupus erythematosus. Several therapies have been attempted with no consensus on an effective therapeutic regimen. Intravenous immunoglobulin has been used in severe manifestations of systemic lupus erythematosus and recently in hypocomplementemic urticarial vasculitis. We present a 7-year-old girl with hypocomplementemic urticarial vasculitis associated with systemic lupus erythematosus and pneumonia who responded favorably to intravenous immunoglobulin.

  6. Thrombosis in vasculitis.

    Science.gov (United States)

    Springer, Jason; Villa-Forte, Alexandra

    2013-01-01

    To review the association of thrombosis and vasculitis and discuss some of the proposed causal mechanisms. It is becoming increasingly evident that various systemic inflammatory diseases such as vasculitis are associated with an increased risk of both venous and arterial thrombosis. Increasing evidence supports the use of immunosuppression in the management of venous thrombosis in Behçet's disease. An increased incidence of thromboembolic disease in antineutrophil cytoplasmic antibody-associated vasculitis has been recognized, especially during periods of active disease. In addition, a higher risk of ischemic heart disease in these patients has also been observed. As in giant cell arteritis, recent evidence supports the role of aspirin in the prevention of ischemic events in Takayasu's disease. Thromboembolic disease is an important complication of several forms of systemic vasculitis, and it may result in significant morbidity and mortality. Many questions such as the role for screening of asymptomatic patients, prevention of thrombosis, and duration of anticoagulation in patients with vasculitis remain unanswered. Future studies exploring the mechanisms of thrombosis and its link to inflammation may provide insights in predicting patients at a higher risk for thrombosis and improve outcomes.

  7. Thromboangiitis obliterans with multiple large vessel involvement: case report and analysis of immunophenotypes.

    Science.gov (United States)

    Edo, Naoki; Miyai, Kosuke; Ogata, Sho; Nakanishi, Kuniaki; Hiroi, Sadayuki; Tominaga, Susumu; Aiko, Satoshi; Kawai, Toshiaki

    2010-01-01

    Thromboangiitis obliterans (TAO, Buerger's disease) is an idiopathic, recurrent, segmental, nonatherosclerotic, inflammatory, occlusive vascular disease with a poorly understood pathogenesis. Intestinal or multi-organ involvement is rare. Recent immunohistochemical analyses of ordinary TAO have indicated an inflammatory and immunologic pathogenesis. We report a case of TAO involving multiple large vessels. By immunohistochemistry, CD3+ T cells were revealed around the recanalization sites within the abdominal aorta. CD4+ T cells were almost equal in number to CD8+ T cells. These findings indicate the participation of inflammatory and immunologic processes in TAO with multi-organ involvement (as in ordinary TAO).

  8. Thoracic vasculitis presenting as surgical problems.

    LENUS (Irish Health Repository)

    Jansen, Michael

    2010-01-01

    We present four patients with vasculitis manifesting with unusual clinical or pathological features, generating surgical problems. Two cases presented with pulmonary hypertension, with investigations and radiological evidence prompting clinical suspicion of pulmonary thrombo-embolic disease. First case, with an antecedant history of Wegener\\'s granulomatosis (WG), demonstrated following "embolectomy", WG involving the large pulmonary elastic arteries. The second case of inoperable "pulmonary thrombo-embolic disease" was subsequently found at limited post mortem to have giant cell arteritis, which affected widespread small peripheral pulmonary arterial vessels. The other two cases were of aortitis occurring in the background of immune-mediated disease, which had been treated with aggressive immunosuppression regimens. The first of these was a case of Cogan\\'s syndrome complicated by descending aortitis, a rarely reported phenomenon, with co-existent acute endocarditis of the aortic valve leaflets. Most cases of endocarditis in this context occur secondary to and in continuity with ascending aortitis. That this case, and a case of ascending aortitis occurring in the context of relapsing polychondritis occurred in the face of aggressive immunosuppression with an apparent clinical response, underscores the need to not accept a clinical picture at face value. This has implications for clinical management, particularly in the follow-up of surgical prosthetic devices such as grafts which may be used in these cases. All four cases emphasise the continued importance of histology and the post-mortem examination in elucidating previously undetected or unsuspected disease.

  9. Actemra Approved for Certain Blood Vessel Inflammation

    Science.gov (United States)

    ... 165836.html Actemra Approved for Certain Blood Vessel Inflammation Drug will treat adults with a condition called ... to treat adults with giant cell arteritis, an inflammation of the blood vessels (vasculitis). In a media ...

  10. Anti-TNF-Alpha Therapy and Systemic Vasculitis

    Directory of Open Access Journals (Sweden)

    Pierre-André Jarrot

    2014-01-01

    Full Text Available TNF-α is a pleiotropic cytokine, which plays a major role in the pathogenesis of numerous autoimmune and/or inflammatory systemic diseases. Systemic vasculitis constitutes a group of rare diseases, characterized by inflammation of the arterial or venous vessel wall, causing stenosis and thrombosis. Treatment of the different type of vasculitis mainly relies on steroids and immunosuppressive drugs. In case of refractory or relapsing diseases, however, a second line of treatment may be required. Anti-TNF-α drugs have been used in this setting during the last 15 years with inconsistent results. We reviewed herein the use of anti-TNF-α therapy in different kind of vasculitis and concluded that, except for Behcet’s disease, this therapeutic option has not demonstrated significant improvement in the treatment of vasculitis.

  11. [Vasculitis and viral infection].

    Science.gov (United States)

    Martínez Aguilar, N E; Guido Bayardo, R; Vargas Camaño, M E; Compañ González, D; Miranda Feria, A J

    1997-01-01

    Viruses have been implicated in vasculitis. To determine activity of viral infection associated with vasculitis. 17 patients with vasculitis had been in immunological and antiviral antibodies evaluation. Twenty five healthy controls sex and age matched with hematic biometry (BH) and AA. All subjects were negative to HIV and HBV. Viral activity was demonstrated in eight patients; vascular purpura (5), Takayasu disease (1), polyarteritis nodosa (1), erythema nodosum (1). None subject of control group had IgM activity. Antibodies response of IgG in patients were of lesser intensity than in control group. 14 abnormalities in BH were found in patients and 4 in control group. Immune response in patients, measured by lymphocyte subpopulations and circulating immune complexes was abnormal. In conclusion 47% showed viral activity, but the dominant feature was abnormal immune response in 82%.

  12. Therapeutic advances in the treatment of vasculitis.

    Science.gov (United States)

    Eleftheriou, Despina; Brogan, Paul A

    2016-04-26

    Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trials involving children for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis; and identification of monogenic forms of vasculitis that provide an understanding of pathogenesis, thus facilitating more targeted treatment. Robust randomised controlled trials for Henoch Schönlein Purpura nephritis and Takayasu arteritis are needed; there is also an over-arching need for trials examining new agents that facilitate corticosteroid sparing, of particular importance in the paediatric population since glucocorticoid toxicity is a major concern.

  13. Hypocomplementemic urticarial vasculitis syndrome in three siblings.

    Science.gov (United States)

    Ozçakar, Z Birsin; Yalçınkaya, Fatoş; Altugan, F Semsa; Kavaz, Aslı; Ensari, Arzu; Ekim, Mesiha

    2013-03-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is relatively uncommon and generally seen in the fourth decade of life. There are very few pediatric cases with the diagnosis of HUVS in the literature. In this report, we describe the first familial cases of HUVS in three siblings. The disease onset was during childhood period in all patients. One of them developed severe renal involvement and died. The other two had ongoing skin and eye manifestations and the elder one developed lupus. Presence of these three patients is a strong evidence for the role of genetic factors in the pathogenesis of this rare vasculitis.

  14. A man with small vessel vasculitis presenting with brachial diplegia, multiple cranial mononeuropathies and severe orthostatic hypotension in diabetes mellitus: a case report.

    Science.gov (United States)

    Zafar, Sahar F; Goodman, Jerry Clay; Ubogu, Eroboghene E

    2013-10-01

    We report a rare case of fulminant vasculitic mononeuropathy resulting in brachial diplegia, with suspected brainstem and autonomic nervous system involvement in a patient with diabetes mellitus. A 58-year-old Hispanic Caucasian man with diabetes mellitus presented with a 1-year history of progressive bilateral upper extremity weakness, orthostatic intolerance and progressive memory decline. Diagnostic evaluation including laboratory tests for progressive encephalopathies, systemic inflammatory and non-inflammatory neuropathies, cerebrospinal fluid analyses, electrodiagnostic studies, and nerve biopsy were performed. Clinical examination revealed moderate cognitive deficits on the Montreal Cognitive Assessment scale, bilateral facial weakness and weakness of bilateral shoulder girdle and intrinsic hand muscles. Cerebrospinal fluid analyses revealed elevated protein and an elevated immunoglobulin G synthesis rate, suggesting an immune-mediated process. Further laboratory work up was non-diagnostic. Electrodiagnostic studies demonstrated chronic asymmetric axonal mononeuropathies with ongoing denervation. A superficial radial nerve biopsy showed a chronic vasculitic neuropathy. Glucocorticosteroid treatment, symptomatic pharmacologic and supportive non-pharmacologic therapies resulted in improved clinical outcomes despite challenges with glycemic control. This case report emphasizes the importance of a thorough evaluation of atypical or uncommon neuromuscular presentations in diabetic patients without etiological presumptions. This is necessary in order to promptly establish a diagnosis, initiate appropriate therapies and prevent irreversible nerve injury.

  15. Large vessel vasculitides

    OpenAIRE

    Morović-Vergles, Jadranka; Pukšić, Silva; Gudelj Gračanin, Ana

    2013-01-01

    Large vessel vasculitis includes Giant cell arteritis and Takayasu arteritis. Giant cell arteritis is the most common form of vasculitis affect patients aged 50 years or over. The diagnosis should be considered in older patients who present with new onset of headache, visual disturbance, polymyalgia rheumatica and/or fever unknown cause. Glucocorticoides remain the cornerstone of therapy. Takayasu arteritis is a chronic panarteritis of the aorta ant its major branches presenting commonly in y...

  16. Urticarial vasculitis secondary to paroxetine.

    Science.gov (United States)

    Welsh, John Patrick; Cusack, Carrie Ann; Ko, Christine

    2006-01-01

    Paroxetine (Paxil) is a widely prescribed antidepressant of the selective serotonin reuptake inhibitor (SSRI) class for which multiple cutaneous adverse effects have been reported, including urticaria, ecchymoses, vasculitis, and pruritus. In contrast, fluoxetine (Prozac) is the only SSRI previously reported to cause urticarial vasculitis. Urticarial vasculitis is a chronic disorder marked by recurrent episodes of erythematous, indurated wheals that histologically manifest the features of leukocytoclastic vasculitis. This case represents the first reported incidence of paroxetine-induced urticarial vasculitis, and highlights the need to consider the entire SSRI class as s as a potential cause of the condition.

  17. Refractory urticarial vasculitis as a complication of ulcerative colitis successfully treated with rituximab.

    Science.gov (United States)

    Swaminath, Arun; Magro, Cynthia M; Dwyer, Edward

    2011-08-01

    Ulcerative colitis can be complicated by the development of leukocytoclastic vasculitis, a cutaneous vasculitis with the potential for systemic involvement. We present a man with a history of ulcerative colitis complicated by end-stage liver disease secondary to sclerosing cholangitis requiring a liver transplant. The patient developed new-onset vasculitis and diarrhea refractory to therapy with standard immunosuppression. He was treated with anti-CD20 therapy with a positive response. The basis of the vasculitis was likely one related to an underlying monoclonal paraprotein with cryoprecitable properties. Treatment with anti-B-cell therapy may be a new treatment option for patients with gammopathy-associated leukocytoclastic vasculitis.

  18. The European Vasculitis Society 2016 Meeting Report

    Directory of Open Access Journals (Sweden)

    Ingeborg M. Bajema

    2017-11-01

    Full Text Available The 2016 European Vasculitis Society (EUVAS meeting, held in Leiden, the Netherlands, was centered around phenotypic subtyping in antineutrophil cytoplasmic antibody (ANCA–associated vasculitis (AAV. There were parallel meetings of the EUVAS petals, which here report on disease assessment; database; and long-term follow-up, registries, genetics, histology, biomarker studies, and clinical trials. Studies currently conducted will improve our ability to discriminate between different forms of vasculitis. In a project that involves the 10-year follow-up of AAV patients, we are working on retrieving data on patient and renal survival, relapse rate, the cumulative incidence of malignancies, and comorbidities. Across Europe, several vasculitis registries were developed covering over 10,000 registered patients. In the near future, these registries will facilitate clinical research in AAV on a scale hitherto unknown. Current studies on the genetic background of AAV will explore the potential prognostic significance of genetic markers and further refine genetic associations with distinct disease subsets. The histopathological classification of ANCA-associated glomerulonephritis is currently evaluated in light of data coming out of a large international validation study. In our continuous search for biomarkers to predict clinical outcome, promising new markers are important subjects of current research. Over the last 2 decades, a host of clinical trials have provided evidence for refinement of therapeutic regimens. We give an overview of clinical trials currently under development, and consider refractory vasculitis in detail. The goal of EUVAS is to stimulate ongoing research in clinical, serological, and histological management and techniques for patients with systemic vasculitis, with an outlook on the applicability for clinical trials.

  19. B cell epitope specificity in ANCA-associated vasculitis : does it matter?

    NARCIS (Netherlands)

    Van der Geld, YM; Stegeman, CA; Kallenberg, CGM

    Pauci-immune idiopathic small-vessel vasculitis is strongly associated with the presence of antineutrophil cytoplasm autoantibodies (ANCA). Antibodies to PR3 predominate in patients with Wegener's granulomatosis; antibodies to myeloperoxidase (MPO) are found more frequently in patients with

  20. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

    Directory of Open Access Journals (Sweden)

    Kae Watanabe

    2016-01-01

    Full Text Available Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors’ knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition.

  1. A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide

    Science.gov (United States)

    Watanabe, Kae; Rajderkar, Dhanashree A.; Modica, Renee F.

    2016-01-01

    Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition. PMID:27018080

  2. [Large vessels vasculopathy in systemic sclerosis].

    Science.gov (United States)

    Tejera Segura, Beatriz; Ferraz-Amaro, Iván

    2015-12-07

    Vasculopathy in systemic sclerosis is a severe, in many cases irreversible, manifestation that can lead to amputation. While the classical clinical manifestations of the disease have to do with the involvement of microcirculation, proximal vessels of upper and lower limbs can also be affected. This involvement of large vessels may be related to systemic sclerosis, vasculitis or atherosclerotic, and the differential diagnosis is not easy. To conduct a proper and early diagnosis, it is essential to start prompt appropriate treatment. In this review, we examine the involvement of large vessels in scleroderma, an understudied manifestation with important prognostic and therapeutic implications. Copyright © 2015 Elsevier España, S.L.U. All rights reserved.

  3. Management of ANCA-associated vasculitis: Current trends and future prospects

    Directory of Open Access Journals (Sweden)

    Sally Hamour

    2010-06-01

    Full Text Available Sally Hamour, Alan D Salama, Charles D PuseyImperial College Kidney and Transplant Institute, Imperial College, London, UKAbstract: The antineutrophil cytoplasm antibody (ANCA-associated vasculitides are a spectrum of heterogeneous autoimmune diseases characterized by necrotizing small vessel vasculitis and the presence of ANCA. These chronic multisystem disorders may be life-threatening if there is major organ involvement, such as acute renal failure or pulmonary hemorrhage, and require significant initial immunosuppression and long-term maintenance treatment. Long-established protocols using cyclophosphamide and prednisolone have resulted in dramatically improved outcomes for patients since the 1970s. Subsequently, international collaboration has contributed to a growing evidence base and consensus in the management of these rare disorders. Modifications to traditional treatment protocols by the use of azathioprine or methotrexate rather than cyclophosphamide, and the introduction of newer agents, such as rituximab, has maintained outcomes whilst decreasing toxicity. However, the treatment limitations of incomplete efficacy, infection, and cumulative toxicity persist. These issues have continued to drive the search for safer and more effective modulation of the immune system using targeted immunotherapy. This review will explore the current evidence base for management of ANCA-associated vasculitis and future treatment prospects.Keywords: ANCA, vasculitis, treatment

  4. A Case of Crescentic Glomerulonephritis Complicated with Hypocomplementemic Urticarial Vasculitis Syndrome and ANCA-Associated Vasculitis

    Directory of Open Access Journals (Sweden)

    Marenao Tanaka

    2017-11-01

    Full Text Available Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively. Complement analysis showed that levels of total hemolytic component, component C3 fraction, and component C4 fraction were 30∼60% of normal values and the titer of anti-neutrophil cytoplasmic antibody for myeloperoxidase (MPO-ANCA was 89 EU (normal range, <10 EU, though there were no immunologic disorders such as systemic lupus erythematosus. Cellular crescentic glomerulonephritis was observed by light microscopy, and immunofluorescent studies showed positive staining for IgG, IgM, C3, C4, and C1q. Electron microscopy showed mesangial and subendothelial deposits with circumferential mesangial interposition. She fulfilled the diagnostic criteria for hypocomplementemic urticarial vasculitis syndrome (HUV, and ANCA-associated vasculitis (AAV was also indicated by small vessel vasculitis and positive MPO-ANCA. Steroid pulse therapy with methylprednisolone followed by oral prednisolone improved her general condition and hypocomplementemia, and MPO-ANCA became negative. HUV and AAV are distinct clinical disorders, though both affect small blood vessels. Here we report a case of AAV-complicated HUV with crescentic glomerulonephritis.

  5. Telmisartan induced urticarial vasculitis.

    Science.gov (United States)

    Mahajan, Vikram K; Singh, Ravinder; Gupta, Mrinal; Raina, Rashmi

    2015-01-01

    A 53-year-old man developed urticarial vasculitis following ingestion of telmisartan and hydrochlorothiazide combination for hypertension. Treatment with prednisolone and cetirizine was curative, but his lesions recurred when he continued telmisartan and hydrochlorothiazide against medical advice. Re-challenge with the same doses of telmisartan precipitated similar lesions with telmisartan and not with hydrochlorothiazide. This uncommon cutaneous adverse reaction of angiotensin II receptor blockers has implication for the clinicians as more such cases may become apparent with their wider use than in premarketing studies.

  6. Retinal vasculitis revealing immunoglobulin G subclass deficiency.

    Science.gov (United States)

    Hassani, Rachid Tahiri Joutei; Rousseau, Antoine; de Monchy, Ivan; el Sanharawi, Mohamed; Gendron, Gael; Barreau, Emmanuel; Goujard, Cécile; Labetoulle, Marc

    2013-01-01

    Immunoglobulin G (IgG) subclass deficiency is a rare primary immunodeficiency syndrome characterized by recurrent infections and autoimmune disorders. However, there have been no reports of ocular involvement, either inflammatory or infectious, in association with IgG subclass deficiency. The authors report the first case of retinal vasculitis that led to the diagnosis of IgG subclass deficiency, in a patient with a history of inflammatory bowel disease and recurrent infections of previously unknown origin.

  7. Severe food-induced vasculitis in two children.

    Science.gov (United States)

    Businco, Luisa; Falconieri, Paola; Bellioni-Businco, Barbara; Bahna, Sami L

    2002-02-01

    Food-induced vasculitis seems to be rare and is considered by some as controversial. The reported cases in the literature are few and mostly on adult patients. Described in this report are two children with severe vasculitis caused by specific foods. They were diagnosed at two separate allergy centers that have a special interest in food allergies. Case 1 was an 8-year-old girl with a 9-month history of cutaneous vasculitis with large joints involvement. Case 2 was a 23-month-old girl with an 8-month history of multiple hospitalizations for recurrent acute severe cutaneous and mucous membrane vasculitis with large joints involvement. In both patients, skin biopsy showed leucocytoclastic vasculitis. In neither of the patients could the symptoms be attributed to drug intake, infection, autoimmunity, or other systemic disease. Case 1 had a moderately elevated serum total immunoglobulin E (IgE) level and strongly positive skin test and radioallergosorbent test (RAST) to cow's milk and hen's egg, both of which were proven to be the cause by elimination-challenge tests. Case 2 had a slightly elevated serum total IgE level, but negative skin tests to foods, including chocolate that was suspected by the mother. Avoidance of chocolate resulted in remission, except following accidental ingestion of cocoa-containing products. These findings support the few previous reports on food-induced vasculitis, an entity that seems rare but may be more common than currently realized.

  8. Hypocomplementemic urticarial vasculitis in systemic lupus erythematosus.

    Science.gov (United States)

    Her, Min Young; Song, Joo Yeon; Kim, Dong Yook

    2009-02-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

  9. Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

    OpenAIRE

    Her, Min Young; Song, Joo Yeon; Kim, Dong Yook

    2009-01-01

    Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

  10. Behcet's disease involving the breast

    Energy Technology Data Exchange (ETDEWEB)

    Soleto, Maria Jesus; Marcos, Lourdes [Department of Radiology, Hospital de la Princesa, Universidad Autonoma, Diego de Leon 62, 28006 Madrid (Spain)

    2002-07-01

    Behcet's disease is a vasculitis of unknown origin that was traditionally defined by oral and genital ulcers and uveitis. We describe a case of a patient with a diagnosis of Behcet's syndrome who presented a palpable lesion in the right breast with inflammatory signs. X-ray findings posed a differential diagnosis between tumoral and inflammatory pathology. The pathological findings confirmed a small-vessel vasculitis. We found two reports of breast involvement by this disease in the literature. Our patient was studied by mammogram and sonogram which together with clinical history are important to prevent delay in diagnosis and unnecessary therapeutic procedures. (orig.)

  11. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS)

    DEFF Research Database (Denmark)

    Walsh, Michael; Merkel, Peter A; Peh, Chen Au

    2013-01-01

    Granulomatosis with polyangiitis (GPA, Wegener's) and microscopic polyangiitis (MPA) are small vessel vasculitides collectively referred to as anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). AAV is associated with high rates of morbidity and mortality due to uncontrolled disease...

  12. Urticarial vasculitis: a unique presentation.

    Science.gov (United States)

    Stigall, Landon E; Sigmon, Justin R; Leicht, Stuart S

    2009-05-01

    Urticarial vasculitis is a relatively rare diagnosis in a patient presenting with urticaria. The process is classically described as a generalized eruption, painful more so than pruritic, lasting longer than 24 hours. Two forms of urticarial vasculitis have been described: ahypocomplementemic form more commonly associated with systemic disease, and a normocomplementemic form that is generally limited to the skin. We report on a uniquely distributed vasculitic eruption restricted mainly to the anterior belt line area in a patient presenting with urticaria and intense pruritus. Urticarial vasculitis as a unique entity is reviewed along with its clinical and histopathologic presentation and the pharmacologic agents used for treatment.

  13. Urticarial vasculitis in northern Spain: clinical study of 21 cases

    National Research Council Canada - National Science Library

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A; Alvarez, Lino; González-Vela, M Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A; Blanco, Ricardo

    2014-01-01

    Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis...

  14. Vasculitis integrated clinical assessment database: a data management system to support studies into systemic vasculitis.

    Science.gov (United States)

    Wilson, Andrew S; Bacon, Paul A; Young, Stephen P; Carruthers, David M

    2010-01-01

    The systemic vasculitides are characterized by immune inflammation affecting blood vessels, which can lead to organ and tissue damage. Treatment has improved but optimum long-term management still remains unsatisfactory, requiring ongoing therapeutic studies. These often base their measures of efficacy on the outcome of clinical assessments which include the Birmingham Vasculitis Activity Score and the Vasculitis Damage Index. Efficient management of assessment data is complex and often hampered by working with time-consuming paper-based systems. The Vasculitis Integrated Clinical Assessment Database (VICAD) was created to improve the process. VICAD was developed using Microsoft Access. Visual Basic for Applications and the Data Access Objects Application Programming Interface provide the functionality to assist with scoring, calculation of results, and storing of data. VICAD is an efficient system for managing data. Evaluation of its use showed an improvement in the completeness of patient assessments from 77% (paper based: n = 44) to 98% (computer based: n = 30). During development clinicians (n = 4) rated it at 8/10 for its layout and visual presentation and 8/10 for easy to use (intuitiveness and navigability). The development of an integrated and standardized system of data collection (VICAD) helps to support clinical decision making processes and report findings in a more timely manner. It is available free for use and modification under the GNU General Public License. The open source nature of VICAD could help to inform the design of other databases where management of complex information into important multisystem diseases is needed.

  15. Kidney involvement in a Wegener granulomatosis case.

    Science.gov (United States)

    Cavoli, Gioacchino Li; Ferrantelli, Angelo; Bono, Luisa; Tortorici, Calogera; Passantino, Rita; Rotolo, Ugo

    2012-01-01

    Wegener Granulomatosis is a systemic Anti-Neutrophil Cytoplasmic Autoantibody-associated Vasculitis, affecting small-to-medium vessels. Clinical presentation with simultaneous involvement of kidney and upper and lower respiratory tract is unusual. We report an instructive case of WG, analyzing clinical course, laboratory, and radiological features, kidney, lung, and larynx histological pictures. Besides renal biopsy, nephrology team performed larynx and lung biopsies because of unusual clinical presentation, computed tomography chest examination, and relevant malignancy risk regarding following immunosuppressant therapy.

  16. Urticarial vasculitis and Schnitzler syndrome.

    Science.gov (United States)

    Zuberbier, Torsten; Zuberbier, H C Torsten; Maurer, Marcus

    2014-02-01

    Both urticarial vasculitis and Schnitzler syndrome are serious diseases but a recently gained better understanding of their underlying pathomechanism allows better treatment options than only a decade ago. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Vancomycin-Associated Leukocytoclastic Vasculitis

    Directory of Open Access Journals (Sweden)

    Makhawadee Pongruangporn

    2011-01-01

    Full Text Available Vancomycin is U.S. Food and Drug Administration (FDA approved for treatment of serious infections caused by methicillin-resistant Staphylococcus aureus (MRSA or in individuals who have failed, cannot tolerate, or are allergic to other antibiotics. Very few cases of vancomycin-associated leukocytoclastic vasculitis have been published. We report on a patient who developed pruritus and palpable purpura in both lower extremities after receiving six days of intravenous vancomycin. Skin biopsy revealed leukocytoclastic vasculitis.

  18. A reassessment of diagnostic criteria and treatment of idiopathic urticarial vasculitis: a retrospective study of 47 patients.

    Science.gov (United States)

    Tosoni, C; Lodi-Rizzini, F; Cinquini, M; Pasolini, G; Venturini, M; Sinico, R A; Calzavara-Pinton, P

    2009-03-01

    Urticarial vasculitis (UV) is an uncommon type of chronic urticaria (CU), which exhibits leucocytoclastic vasculitis. Painful and long-lasting (> 24 h) weals associated with purpura or bruising are considered indicative of UV. It is often responsive to oral corticosteroids and poorly to oral antihistamines. Hypocomplementaemia and systemic involvement are also commonly reported. To diagnose patients with UV histologically and then compare their clinical features and response to various treatment regimens. Biopsies were taken from 312 subjects with CU unresponsive to oral antihistamines; of these, 47 were histologically diagnosed as having UV. Biopsies were taken irrespective of the clinical features of weal eruption. Other diseases known to be associated with small-vessel vasculitis had previously been excluded. Results. Individual weals lasted < 24 h in 57.4% of patients, and pain or tenderness was reported only by 8.6%. Extracutaneous features were present in 81%, hypocomplementaemia in 11% and abnormalities of other laboratory parameters (i.e. raised erythrocyte sedimentation rate, microscopic haematuria) in 76.6%. Hydroxyzine was effective in only one patient. Both oral corticosteroids and cinnarizine were effective in a high percentage of the patients. This diagnostic approach allowed us to identify a large group (47 patients) with UV. Most did not present the clinical (prolonged duration of weals and bruising) and laboratory features that have previously been described as characteristic of UV. Cinnarizine was found to be a valuable treatment option.

  19. Imaging of CNS Vasculitis

    OpenAIRE

    Nahid Sedighi

    2010-01-01

    The vasculitides are the most interesting assorted group of diseases. Most have an imunologic basis resulting in vascular injury."nInflammatory changes within and surrounding the vessel wall result in narrowing and obliteration of the vascular lumen with subsequent thrombotic occlusion or necrosis and rupture of the vessel. The pathologic features in this lesion include spasm, edema, cellular infiltration, and proliferation. "nThese inflammatory changes result in the classic angiogr...

  20. Unihemispheric central nervous system vasculitis

    Directory of Open Access Journals (Sweden)

    Sikawat Thanaviratananich

    2017-06-01

    Full Text Available Patients with primary central nervous system vasculitis (PCNSV usually manifest with multiple enhancing bilateral hemispheric lesions. We presented an extremely rare clinical course and follow-up of a patient with PCNSV affecting only a single (right hemisphere. A 33-year-old previously healthy man presented with a left hand clonic seizure followed by a secondary generalized tonic-clonic seizure and dysarthria. MRI brain revealed multiple hyperintense lesions confined to only the right hemisphere with contrast enhancement, involving both white and grey matters. He was treated with a methylprednisolone for 5 days followed by prednisone for suspected acute disseminated encephalomyelitis without improvements. He was presented again with left-sided weakness, transient dysarthria and black objects in left visual field. MRI brain was unchanged. MR angiogram and conventional cerebral angiogram were normal. Autoimmune work-ups were all negative. A brain biopsy showed evidence of PCNSV. He was then successfully treated with intravenous cyclophosphamide followed by oral azathioprine. On a follow-up 3 years later, he remains asymptomatic on azathioprine and a repeat MRI showed all areas of enhancement were gone.

  1. Churg-Strauss syndrome and hemorragic vasculitis

    Directory of Open Access Journals (Sweden)

    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.

  2. Current status of outcome measure development in vasculitis.

    Science.gov (United States)

    Merkel, Peter A; Aydin, Sibel Z; Boers, Maarten; Cornell, Christina; Direskeneli, Haner; Gebhart, Don; Hatemi, Gulen; Luqmani, Raashid; Matteson, Eric L; Milman, Nataliya; Robson, Joanna; Seo, Philip; Tomasson, Gunnar

    2014-03-01

    The conduct of randomized controlled trials for vasculitis, especially for the antineutrophil cytoplasmic antibody-associated vasculitides [AAV, granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis], has been greatly advanced by the development, use, and acceptance of validated outcome measures. Trials have subsequently provided the opportunity to validate and refine reliable, valid outcome measures for these multisystemic and relapsing rare diseases. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group was formed in 2004 to foster development of validated and widely accepted outcomes in vasculitis using data-driven analyses, a dedication to building consensus, and adherence to, and guidance by, the principles of the OMERACT approach. This work led to the endorsement by OMERACT of the core set of domains and associated outcome measures for AAV. Next steps for the study of existing outcome tools in AAV include better definition of response criteria through development of more data-driven weighting of the elements of activity and damage assessment. The Working Group is now also embarking on a series of linked projects to develop validated patient-reported outcomes for use in clinical research in vasculitis. Additionally, the Working Group is studying how current methods of disease assessment and plans for new outcomes can be informed by the conceptual framework of the International Classification of Function of the World Health Organization. The success of the Group's work in AAV has also led to a formal process for developing outcomes for the large vessel vasculitides (Takayasu arteritis and giant cell arteritis) and Behçet disease.

  3. 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

  4. [Uptodate in the management and treatment of ANCA-associated vasculitis].

    Science.gov (United States)

    Belaconi, Ionela Nicoleta; Toma, Claudia Lucia; Bogdan, Miron Alexandru

    2014-01-01

    The antineutrophil cytoplasm antibody (ANCA)-associated vasculitis are heterogeneous, multisystem, autoimmune diseases characterized by necrotizing small and medium vessel vasculitis and the association with ANCA. The diagnosis and management of these patients may be challenging due to the variability of clinical features, the possibility of life-threatening events (acute renal failure or pulmonary hemorrhage) and the relative rarity of these syndromes. ANCA-associated vasculitis include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. The treatment requires significant immunosuppression and there are frequent treatment related side effects. Although the standard protocol with cytotoxic agents and glucocorticoids has dramatically improved patient outcome, its toxic profile remains a major problem. Recent progress in evidence base and consensus in understanding the pathogenic mechanism and the quantification of disease activity further improved patient's life. Special attention was paid in refining immunosuppressive treatment to minimize his toxicity. This review will focus on evidence based treatment of ANCA-associated vasculitis.

  5. Urticarial vasculitis reveals unsuspected thyroiditis.

    Science.gov (United States)

    Ferreira, Olga; Mota, Alberto; Baudrier, Teresa; Azevedo, Filomena

    2012-01-01

    A 38-year-old woman presented with erythematous, violaceous plaques with a serpiginous and unusual appearance located on the left shoulder, left thigh, and right buttock, evolving for 5 days, which eventually became generalized. A skin biopsy revealed leukocytoclastic vasculitis and a diagnosis of urticarial vasculitis was made. The complete blood count, biochemistry, complement levels, and other immunological test results were unremarkable. However, antithyroid antibody titers were increased. Despite having normal thyroid function tests and an absence of specific symptoms, the patient underwent a thyroid ultrasound, which revealed features of thyroiditis, and was subsequently referred to an endocrinologist. Several diseases can be associated with urticarial vasculitis, namely infections and autoimmune connective-tissue disorders such as systemic lupus erythematosus and Sjögren syndrome. Thyroiditis is an uncommon association.

  6. Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report.

    Science.gov (United States)

    Jung, Su Woong; Choi, Yun Young; Choi, In Seung; Kim, Seulki; Jeong, Kyung Hwan; Song, Ran; Lee, Sang Hoon; Yang, Hyung In; Hong, Seung Jae; Lee, Yeon Ah

    2017-12-01

    Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarial vasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards, HUVS was systemically complicated with myositis and membranous nephropathy that developed several months and about 2 years after its onset, respectively. A combination of glucocorticoid and immunosuppressive agents has been used to effectively control disease activity. © 2017 The Korean Academy of Medical Sciences.

  7. Spontaneous abdominal hemorrhage with AA-amyloidosis and vasculitis in a patient with rheumatoid arthritis.

    Science.gov (United States)

    Jayawardene, S A; Sheerin, N; Pattison, J M; Hartley, B; Goldsmith, D J

    2001-04-01

    Both rheumatoid vasculitis and amyloidosis in rheumatoid arthritis (RA) are uncommon. We describe a patient in whom they occurred together and were associated with fatal intra-abdominal hemorrhage. A 56-year-old Caucasian woman was referred because of increasing lethargy, edema, and proteinuria. She had suffered from seropositive, erosive, nodular RA for 14 years. Previously, she had undergone numerous joint replacements, a thyroidectomy for amyloid-associated (AA) amyloidosis of the thyroid that caused a large goiter and a renal biopsy that showed renal AA-amyloidosis in the context of nephrotic syndrome. As her condition deteriorated, this patient became increasingly reluctant to go to the hospital and to take drugs beyond analgesics. Thus, her RA was chronically under treated. While in the hospital for evaluation, this patient suddenly developed hypotension, tachycardia, and a severe colicky left-sided abdominal pain radiating from the left upper quadrant/epigastric region to the left iliac fossa. Computed tomography (CT) showed a large amount of echogenic free fluid within the abdomen and marked thickening of the omentum. At laparotomy, 2 liters of free blood was found adjacent to a hematoma of the greater omentum, and it was evacuated without identification of a discrete bleeding point. All solid and hollow organs were normal. The omentum was noted to be very friable. She developed a more disseminated bleeding diathesis and persistent peritoneal hemorrhage via her abdominal drains. She succumbed shortly afterward. Histology revealed extensive omental hemorrhage and one large vessel within the area of hemorrhage showed a severe necrotizing vasculitis. Extensive amyloid deposition was also found within the walls of the smaller omental arterioles. Vasculitis in the context of RA is relatively rare and is associated with under treated, seropositive disease. Skin and nerve involvement are most common, but bowel involvement has been reported, with a highly

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

    NARCIS (Netherlands)

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

    Background: 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. Objective: We analysed the diagnostic performance of two rapid ANCA and

  9. Vasculites pulmonares: novas visões de uma velha conhecida Pulmonary forms of vasculitis: new perspectives on an old acquaintance

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    Thais Thomaz Queluz

    2005-07-01

    Full Text Available A vasculite necrosante foi descrita em 1866 e seu espectro é muito amplo, uma vez que acomete vasos arteriais e venosos de todos os calibres e de vários órgãos, apresenta diversos tipos de infiltrados inflamatórios, tem um significante número de manifestações clínicas e pode ter ou não fatores desencadeantes identificáveis. A sempre controversa classificação das vasculites mudou radicalmente com a descoberta dos anticorpos anticitoplasma de neutrófilos em 1982, contemplando atualmente a doença de Goodpasture, as vasculites associadas aos anticorpos anticitoplasma de neutrófilos, as vasculites por imunocomplexos e outros tipos de vasculites. As evidências de que os anticorpos anticitoplasma de neutrófilos estão envolvidos na patogênese destas lesões trouxeram avanços consideráveis para o seu diagnóstico e tratamento. Granulomatose de Wegener, doença de Churg-Strauss e poliangeíte microscópica, todas vasculites associadas aos anticorpos anticitoplasma de neutrófilos, são as vasculites sistêmicas que mais acometem os pulmões. Suas manifestações clínicas comuns são tosse, hemorragia alveolar difusa ou asma de difícil controle. Na arterite de Takayasu, na doença de Behçet, na púrpura de Henoch-Schönlein e nas vasculites associadas às doenças do colágeno o acometimento pulmonar é mais raro. Em todos os casos há evidências de serem processos de origem imunológica e com base neste princípio são propostas as abordagens terapêuticas.Necrotizing vasculitis was first described in 1866. The condition encompasses a wide spectrum of symptoms, affecting arterial blood vessels of various calibers and in various organs. In addition, it is associated with many types of inflammatory infiltrate, and presents a significant number of clinical manifestations. The causative factor or factors may or may not be identifiable. The eternally controversial classification of the various forms of vasculitis changed radically

  10. Involvement of histamine in endothelium-dependent relaxation of mesenteric lymphatic vessels.

    Science.gov (United States)

    Nizamutdinova, Irina Tsoy; Maejima, Daisuke; Nagai, Takashi; Bridenbaugh, Eric; Thangaswamy, Sangeetha; Chatterjee, Victor; Meininger, Cynthia J; Gashev, Anatoliy A

    2014-10-01

    The knowledge of the basic principles of lymphatic function, still remains, to a large degree, rudimentary and will require significant research efforts. Recent studies of the physiology of the MLVs suggested the presence of an EDRF other than NO. In this study, we tested the hypothesis that lymphatic endothelium-derived histamine relaxes MLVs. We measured and analyzed parameters of lymphatic contractility in isolated and pressurized rat MLVs under control conditions and after pharmacological blockade of NO by L-NAME (100 μM) or/and histamine production by α-MHD (10 μM). Effectiveness of α-MHD was confirmed immunohistochemically. We also used immunohistochemical labeling and Western blot analysis of the histamine-producing enzyme, HDC. In addition, we blocked HDC protein expression in MLVs by transient transfection with vivo-morpholino oligos. We found that only combined pharmacological blockade of NO and histamine production completely eliminates flow-dependent relaxation of lymphatic vessels, thus confirming a role for histamine as an EDRF in MLVs. We also confirmed the presence of HDC and histamine inside lymphatic endothelial cells. This study supports a role for histamine as an EDRF in MLVs. © 2014 John Wiley & Sons Ltd.

  11. Meningococal Septicemia with Cutaneous Vasculitis

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    Radha Mittal

    1989-01-01

    Full Text Available Two cases-had meningococcal septicemia with vasculitis of skin. Out 9 year old female developed generalised ,asymptomatic, purpuric atrophic plaques on the limbs, trunk and face on the fourth day of high fever. The second patient was 20 year old male who developed purplish plaques. Nodules, and bullae mainly on the extensor surface of the hands and feet on the tenth day, of high fever. Bullae had clear fluid and bluish-black peripheral rim

  12. Prednisone and vardenafil hydrochloride for refractory levamisole-induced vasculitis.

    Science.gov (United States)

    Mandrell, Joshua; Kranc, Christina L

    2016-08-01

    Levamisole is an immunomodulatory drug that was previously used to treat various medical conditions, including parasitic infections, nephrotic syndrome, and colorectal cancer. Over the last few years, increasing amounts of levamisole have been used as an adulterant in cocaine. Levamisole-cut cocaine has become a concern because it is known to cause a necrotizing purpuric rash, autoantibody production, and life-threatening leukopenia. Mixed histologic findings of vasculitis and thrombosis are characteristic of levamisole-induced purpura. The recommended management of levamisole-induced vasculitis currently involves withdrawal of the culprit along with supportive treatment. We describe a patient with levamisole-induced vasculitis who continued to develop skin lesions despite self-reported cocaine cessation. Complete resolution of cutaneous disease occurred with the addition of oral prednisone and vardenafil hydrochloride, suggesting the possibility of a new treatment option in patients with refractory disease. In addition, we review the clinical presentation, disease course, diagnostic approach, laboratory findings, histology, and management of levamisole-induced vasculitis. The harmful effects of levamisole-cut cocaine are serious enough that public alerts have been issued to increase awareness. Clinicians should consider the possibility of levamisole exposure in cocaine users presenting with any combination of fever, neutropenia, and necrotic skin lesions, especially in acral areas including the ears.

  13. T cell-macrophage interactions and granuloma formation in vasculitis

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    Marc eHilhorst

    2014-09-01

    Full Text Available Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage-T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell-macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy.

  14. Cutaneous leukocytoclastic vasculitis associated with levofloxacin therapy

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    Dana Blyth

    2012-01-01

    Full Text Available Many cases of cutaneous vasculitis are druginduced with histology revealing leukocytoclastic vasculitis (LCV. We present a case of levofloxacin- associated LCV successfully treated with prednisone and cessation of the offending drug. Although case reports describe a link between LCV and older fluoroquinolones, such as ciprofloxacin and ofloxacin, recent reports have implicated the newer fluoroquinolone levofloxacin. Recognition of fluoroquinoloneinduced cutaneous vasculitis is important as continuation or re-exposure of the offending agent may have life-threatening consequences.

  15. Educational Needs of Patients With Systemic Vasculitis

    Science.gov (United States)

    2014-07-11

    Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis

  16. Goodpasture's syndrome associated with pulmonary eosinophilic vasculitis.

    Science.gov (United States)

    Komadina, K H; Houk, R W; Vicks, S L; Desrosier, K F; Ridley, D J; Boswell, R N

    1988-08-01

    Lung hemorrhage and antiglomerular basement membrane (anti-GBM) antibody mediated nephritis define Goodpasture's syndrome. We present the case of a 19-year-old Caucasian woman with unique clinical findings of Goodpasture's syndrome. Our patient initially presented with leukocytoclastic vasculitis of the skin followed by the development of nephritis and lung hemorrhage. An open lung biopsy done prior to diagnosing anti-GBM antibody disease demonstrated an intense eosinophilic vasculitis. Skin vasculitis has only been rarely reported, and to our knowledge this is the first reported case of pulmonary eosinophilic vasculitis associated with Goodpasture's syndrome.

  17. The Vulnerability of Vessels Involved in the Role of Embolism and Hypoperfusion in the Mechanisms of Ischemic Cerebrovascular Diseases

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    Yong Peng Yu

    2016-01-01

    Full Text Available Accurate definition and better understanding of the mechanisms of stroke are crucial as this will guide the effective care and therapy. In this paper, we review the previous basic and clinical researches on the causes or mechanisms of ischemic cerebrovascular diseases (ICVD and interpret the correlation between embolism and hypoperfusion based on vascular stenosis and arterial intimal lesions. It was suggested that if there is no embolus (dynamic or in situ emboli, there might be no cerebral infarction. Three kinds of different clinical outcomes of TIA were theoretically interpreted based on its mechanisms. We suppose that there is a correlation between embolism and hypoperfusion, and which mechanisms (hypoperfusion or hypoperfusion induced microemboli playing the dominant role in each type of ICVD depends on the unique background of arterial intimal lesions (the vulnerability of vessels. That is to say, the vulnerability of vessels is involved in the role of embolism and hypoperfusion in the mechanisms of ischemic cerebrovascular diseases. This inference might enrich and provide better understandings for the underlying etiologies of ischemic cerebrovascular events.

  18. CT findings at lupus mesenteric vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Ko, S.F. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Lee, T.Y. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Cheng, T.T. [Chang Gung Medical College and Memorial Hospital, Dept. of Rheumatology, Kaohsiung Hsien (Taiwan); Ng, S.H. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Lai, H.M. [Chang Gung Medical College and Memorial Hospital, Dept. of Rheumatology, Kaohsiung Hsien (Taiwan); Cheng, Y.F. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan); Tsai, C.C. [Chang Gung Medical College and Memorial Hospital, Dept. of Radiology, Kaohsiung Hsien (Taiwan)

    1997-01-01

    Purpose: To describe the spectrum of early CT findings of lupus mesenteric vasculitis (LMV) and to assess the utility of CT in the management of this uncommon entity. Methods: Abdominal CT was performed within 1-4 days (average 2.2 days) of the onset of severe abdominal pain and tenderness in 15 women with systemic lupus erythematosus. Prompt high-dose i.v. corticosteroid in 11 patients after the CT diagnosis of LMV was made. CT was performed after abdominal symptoms subsided. Results: Eleven cases revealed CT features suggestive of LMV including conspicuous prominence of mesentric vessels with palisade pattern or comb-like appearance (CT comb sign) supplying focal or diffuse dilated bowel loops (n=11), ascites with slightly increased peritoneal enhancement (n=11), small bowel wall thickening (n=10) with double halo or target sign (n=8). Follow-up CT before high-dose steroid therapy revealed complete or marked resolution of the abnormal CT findings. Conclusion: CT is helpful for confirming the diagnosis of LMV, especially the comb sign which may be an early sign. Bowel ischemia due to LMV is less ominous than previously expected, and the abnormal CT findings were reversible when early diagnosis and prompt i.v. steroid therapy could be achieved. (orig.).

  19. A STUDY OF AETIOLOGICAL FACTORS IN THE OCCURRENCE OF CUTANEOUS VASCULITIS IN A TERTIARY CARE CENTRE IN NORTH KERALA

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    Praveen Malayath

    2017-03-01

    Full Text Available BACKGROUND Cutaneous vasculitis is a condition caused by various aetiologies. They can be primary or secondary. Cutaneous lesions maybe a pointer to systemic diseases. So, it is important to identify the various aetiological factors in the occurrence of the various types of cutaneous vasculitis. The patterns and the various aetiologies of cutaneous vasculitis in Kerala is not well documented in the existing literature. MATERIALS AND METHODS The present study is a cross-sectional descriptive study of patients with a clinical diagnosis of cutaneous small vessel vasculitis admitted in Medicine and Dermatology Wards of Calicut, Government Medical College during January 2013 to December 2013. A detailed history and clinical examination of patients along with histopathological examination of skin biopsy was done. RESULTS Out of the 70 cases of cutaneous vasculitis studied, idiopathic cutaneous small vessel vasculitis was the most common type followed by Henoch-Schonlein purpura. The most common aetiology identified was drugs followed by infections. No aetiological factor was identified in 42.8% of the cases. CONCLUSION An aetiological association could be found in 57.8% of cases. The causes identified include drugs, infections, malignancy, connective tissue disorder associated, chronic systemic diseases and Behcet’s disease in decreasing order of frequency.

  20. Testicular Vasculitis: A Sonographic and Pathologic Diagnosis

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    Anuj Dixit

    2017-01-01

    Full Text Available Very little has been published about single-organ vasculitis of the testicle in the radiological literature. Consequently, it is a diagnosis that is unfamiliar to most radiologists. This case report describes the sonographic, pathologic, and laboratory findings of testicular vasculitis and reviews the available literature with regard to this subject.

  1. Urticarial Vasculitis in Northern Spain

    Science.gov (United States)

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A.; Alvarez, Lino; González-Vela, M. Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A.; Blanco, Ricardo

    2014-01-01

    Abstract Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours’ duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1–78 yr; interquartile range, 5–54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2–38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis

  2. Intravascular crystal deposition: an early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

    Science.gov (United States)

    Gammon, Bryan; Longmire, Michelle; DeClerck, Brittney

    2014-09-01

    Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.

  3. Vasculites pulmonares: quando suspeitar e como fazer o diagnóstico Pulmonary vasculitis: when suspicion equal diagnosis

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    Carmen Sílvia Valente Barbas

    2005-07-01

    Full Text Available As vasculites pulmonares primárias são caracterizadas por processo inflamatório na parede dos vasos pulmonares que leva a isquemia e hemorragia pulmonar com as conseqüentes expressões clínicas e radiológicas. As vasculites pulmonares primárias são acompanhadas de expressão sistêmicas cutâneas, em nervos periféricos, rins, seios da face, olhos e ouvidos, além do trato gastrintestinal, e sistemas cardíaco e nervoso central. O diagnóstico é feito através da associação das informações clínicas, radiológicas e anatomopatológicas. O tratamento com corticosteróides e imunossupressores deve ser instituído precocemente e apresenta altas taxas de remissão.The primary forms of pulmonary vasculitis are characterized by an inflammatory process in the pulmonary vessel walls, leading to pulmonary ischemia and hemorrhage and the consequent clinical and radiological manifestations. These forms of vasculitis are accompanied by symptoms involving the skin, peripheral nerves, kidneys, sinuses, eyes, ears and gastrointestinal tract, as well as the cardiac and central nervous systems. The diagnosis is made through analysis of the clinical, radiological and pathological data. When treatment with corticosteroids and immunosuppressive therapy is initiated early, remission rates are high.

  4. D-penicillamine-induced ANA (+) ANCA (+) vasculitis in pediatric patients with Wilson's disease.

    Science.gov (United States)

    Lee, Yeonhee; Lee, Sang Taek; Cho, Heeyeon

    2016-05-01

    Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with systemic vasculitis. The pathophysiology of ANCA-associated vasculitis (AAV) has not been clearly proven, and drug-induced ANCA-associated vasculitis has been reported. Wilson's disease is an inborn error of copper metabolism caused by a mutation in the copper transporting gene ATP7B, and traditional treatment is based on copper chelation with agents such as D-penicillamine. There have been rare reports that prolonged D-penicillamine therapy might cause adverse renal events such as membranous nephropathy and minimal change disease, but it is questionable if D-penicillamine induces ANCA-associated vasculitis. We describe 2 patients with Wilson's disease treated with D-penicillamine who presented with ANCA (+) vasculitis and renal involvement. The 2 patients also showed positive results for antinuclear antibody (ANA). Their kidney biopsy findings were compatible with crescentic/necrotizing glomerulonephritis, pauci-immune type. After diagnosis of AAV, D-penicillamine was stopped. Patients were then treated with plasmapheresis and immunosuppressants, including methylprednisolone pulse therapy and intravenous cyclophosphamide. One patient progressed to end-stage renal disease and the other showed persistent proteinuria. These cases suggest that D-penicillamine may induce ANA (+) ANCA (+) vasculitis with severe renal involvement in pediatric patients, and plasmapheresis combined with immunosuppressant should be considered.

  5. Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India.

    Science.gov (United States)

    Dincy, C V P; George, R; Jacob, M; Mathai, E; Pulimood, S; Eapen, E P

    2008-07-01

    This study aims to study the clinical and histopathological characteristics of hypocomplementemic and normocomplementemic urticarial vasculitis (HUVS and NUV) among dermatology clinic attendees in a tertiary care hospital in South India. A prospective study was conducted in the dermatology department from February 2003 to May 2004. Seventy-five patients met the inclusion criteria for UV. Sixty-eight patients in whom complement levels were available were classified into either NUV or HUVS groups. Clinical features, laboratory parameters and histological features were compared, and the significance of differences was established using Pearson's Chi-squared test. There was a female preponderance among patients with HUVS. Wheals > 24 h were seen in 90% of patients, and in 54.4% of patients, the wheals were partially blanching or non-blanching. Angioedema was more prevalent in patients with NUV than HUVS (44.4% vs. 21.4%). Systemic involvement was seen in 64.3% of patients with HUVS and 44.4% of patients with NUV. Fever, ANA positivity and systemic lupus erythematosus (SLE) were significantly associated with HUVS. In most cases of UV, a provoking factor could not be identified. Neutrophilic small vessel vasculitis was seen in 42.9% of patients with HUVS and 16.6% patients with NUV. Direct immunofluorescence test showing immunoreactants at the dermo-epidermal junction were present in 60% of patients with HUVS and 33.3% patients with NUV. The clinical features of Indian patients with UV were similar to those reported from the West. Fever, ANA positivity and SLE were significantly associated with HUVS.

  6. Infectious urticaria with purpura: a mild subtype of urticarial vasculitis?

    Science.gov (United States)

    Oi, Mieko; Satoh, Takahiro; Yokozeki, Hiroo; Nishioka, Kiyoshi

    2005-01-01

    Urticaria is characterized by transient wheals. We report here five cases with long-lasting urticarial lesions persisting for more than 24 hours. Each lesion left purpura after fading. There was no systemic involvement. C-reactive protein and serum levels of complement were elevated or normal. Histologically, marked infiltration by eosinophils and neutrophils with karyorrhexis in the perivascular and intercollagenous spaces was observed, but there was no evidence of vasculitis (venulitis). Skin symptoms were resistant to systemic corticosteroids. In contrast, treatment of underlying bacterial infections resulted in marked improvement of skin lesions. E-selectin, VCAM-1 and ICAM-1 were expressed on endothelial cells. Marked deposition of C3a, C5a, neutrophil elastase and major basic protein in the dermis was observed. These urticarial lesions provoked by bacterial infections seem to lie on the continuum between urticaria and urticarial vasculitis.

  7. Vasculitis Syndromes of the Central and Peripheral Nervous Systems

    Science.gov (United States)

    ... of the walls of blood vessels in the coronary arteries may cause aneurysms. Symptoms include high fever lasting at least five ... understanding of the mechanisms involved in injury and repair of blood vessels in the brain may lead ...

  8. ANCA-associated vasculitis and malignancy

    DEFF Research Database (Denmark)

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

    2013-01-01

    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...

  9. Evalution of Autoimmunity in Patients with Primary Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Hatice Ozkol

    2014-06-01

    Full Text Available Aim: In order to determine the clinical and etiological characteristics of primer cutaneous vasculitis and to evaluate its relation with immunological mechanisms. Material and Method: Twenty-eight hospitalized patients with cutaneous vasculitis between 2009 -2011 in dermatology service were investigated retrospectively. Patients%u2019 age, gender, disease duration, associated symptoms, infection, and drug history were recorded. Results of immune-fluorescence biopsy and all laboratory tests including detailed antibodies which were investigated for the differential diagnosis of vasculitis were given in percent (% after recording to SPSS 13.0 statistical software. Results: Median age of the 28 patients (18 female, 10 male was 37,89±23,30 (5-84 age years. 39.3% patients had neutrophilia, 67.9% patients had eosinophilia. Antinuclear antibodies (ANA in 10 patients (35.7% were positive. Total IgE in 15 patients (53.6%, IgA in 9 patients (32.1%, IgG in 10 patients (35.7% was higher than normal range. Thyroglobulin in 3 patients (10.7%, antiTPO 4 patients (14.3%, antirubella IgG in 14 patients (50.0% antitoxoplazma IgG in 10 patients (35.7% were positive. Anticardiolipin antibodies, anti-ssa1 and Anti-ssb2, only one patient (3.6% was positive. Discussion: Immunoglobulins and ANA were determined positively in a high ratio in patients with cutaneous leucocytoclastic vasculitis however there was no systemic involvement. Autoimmunity may have an important role in pathogenesis. Moreover drugs were most frequently determined among etiological factors.

  10. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

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    Amel Harzallah

    2017-01-01

    Full Text Available Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

  11. 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.

  12. [Urticarial vasculitis associated with essential thrombocythaemia progressing to myelofibrosis].

    Science.gov (United States)

    Koudoukpo, C; Jachiet, M; Zini, J-M; Andreoli, A; Pinquier, L; Rybojad, M; Bosset, D; de Masson, A; Bagot, M; Lebbé, C; Bouaziz, J-D

    2014-12-01

    Urticarial vasculitis (UV) is a rare form of leukocytoclastic vasculitis in which skin lesions resemble urticaria. UV comprises hypocomplementemic and normocomplementemic subtypes. To date, only 4 cases of UV associated with myeloproliferative disorders have been described, including 3 cases with essential thrombocythaemia (ET) and one case with polycythaemia vera. We describe the case of a 59-year-old male patient with JAK2-positive TE and secondary myelofibrosis and who developed multiple urticarial papules persisting for more than 24hours. Skin biopsy showed perivascular neutrophilic infiltrate with margination of neutrophils in the lumen of vessels and some leukocytoclastic patterns, and with red cell extravasation consistent with UV. Treatment with ruxolitinib (a JAK2 inhibitor) induced transient and partial control of the haematological symptoms but did not prevent UV flare. Prednisolone 20mg once daily was added, with good clinical response. To our knowledge, this is the fourth reported case of UV associated with ET and the first case associated with MF. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  13. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis.

    LENUS (Irish Health Repository)

    Suppiah, Ravi

    2011-05-01

    Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis.

  14. Recurrent optic disc and retinal vasculitis in a patient with drug-induced urticarial vasculitis.

    Science.gov (United States)

    Batioğlu, Figen; Taner, Pelin; Aydintuğ, Olcay Tiryaki; Heper, Aylin Okçu; Ozmert, Emin

    2006-01-01

    The purpose of this study was to report recurrent optic disc and retinal vasculitis in a patient with drug-induced urticarial vasculitis. Complete ophthalmological examination including fluorescein angiography and visual field examination were done. A 53-year-old woman with recurrent painful urticarial skin lesions following trimethoprim sulfamethoxazole usage had the clinical and histopathological diagnosis of urticarial vasculitis. Two years after cutaneous manifestations, she began to notice visual disturbances in both eyes that recurred at 1-year intervals. Her ophthalmological findings were consistent with recurrent vasculitis of the optic nerve and retina. Treatment with high-dose corticosteroids and hydroxychloroquine resulted in the resolution of cutaneous and ocular manifestations. This patient demonstrates that recurrent occlusive vasculitis of the optic nerve and retina can occur in this rare disease. These patients should be examined periodically by ophthalmologists.

  15. Urticarial vasculitis: etiologies and clinical course.

    Science.gov (United States)

    Kulthanan, Kanokvalai; Cheepsomsong, Meethawee; Jiamton, Sukhum

    2009-01-01

    Out of 64 patients diagnosed with urticarial vasculitis (UV), 49 (76.6%) presented with their first attack of UV. The others experienced recurrent attacks with a mean number of 3.3 past recurrences. Fifteen patients had angioedema (23.4%) and 16 (25%) suffered systemic involvement. The most common abnormal laboratory finding was an increased erythrocyte sedimentation rate. Six of 62 patients (9.7%) had decreased C3 levels. A cause could be identified in 19 patients (29.7%). The most common identified cause was infection; other causes included drugs, malignancy and systemic lupus erythematosus (SLE). The prevalence of immunoreactant deposits in the skin lesions measured by DIF was 54.7% (35 of 64 patients). The median disease duration of each episode was 85 days. The probability that patients were free of symptoms within one year was 70%. Patients with an idiopathic cause had a statistically significant longer course duration of each episode than the group with upper respiratory tract infection. Compared to reports from Westem countries, our patients seemed to have less severe symptoms and a lower percentage of hypocomplementemic UV and SLE.

  16. Bilateral Testicular Infarction from IgA Vasculitis of the Spermatic Cords

    Directory of Open Access Journals (Sweden)

    Mazen Toushan

    2017-01-01

    Full Text Available A 51-year-old man with type 2 diabetes mellitus and chronic obstructive pulmonary disease presented to the emergency room with increasing bilateral leg pain, rash, and scrotal swelling with pain. Skin biopsy from his thigh revealed IgA-associated vasculitis. Due to hematuria, a renal biopsy was performed and showed an IgA glomerulonephritis with focal fibrinoid necrosis and neutrophil accumulation. Bilateral orchiectomies were performed in two separate procedures ten and thirteen days after the renal biopsy, as a result of uncontrolled abscess formation in testicles. Microscopically, both testicles revealed large abscess formation destroying almost the entire testicular parenchyma without tumor cells. Spermatic cord margins were further scrutinized microscopically to show bilateral vasculitis in many small size vessels, confirmed by positive endothelial staining for IgA. Some of the affected arteries revealed central organizing thrombi with recanalization features, highly suggestive of vasculitis-associated thrombi formation, resulting in testicular ischemic infarction and abscess formation. We conclude that this adult patient developed a severe form of Henoch-Schönlein purpura, with vasculitis affecting multiple organs, including the most serious and unusual complication of bilateral testicular infarction.

  17. [Acute pancreatitis as the presenting feature of an IgA vasculitis: An unusual presentation].

    Science.gov (United States)

    Fertitta, L; Noel, N; Ackermann, F; Lerolle, N; Benoist, S; Rocher, L; Lambotte, O

    2017-10-01

    IgA vasculitis is a systemic small vessel leukocytoclastic vasculitis characterized by skin purpura, arthritis, abdominal pain and nephritis. Most of the abdominal complications are due to edema and hemorrhage in the small bowel wall, but rarely to acute secondary pancreatitis. Here, we report a 53-year-old woman who presented with acute pancreatitis and, secondarily, developed skin purpura and arthritis at the seventh day of the clinical onset. Biological tests and computed tomographic scan allowed to rule out another cause of pancreatitis and IgA vasculitis was diagnosed as its etiology. The outcome was favorable without any relapse on glucocorticoids. Despite its rarity, pancreatitis is a potential life-threatening complication of IgA vasculitis in which the role of glucocorticoids and immunosuppressive drugs remains uncertain. A prompt elimination of other usual pancreatitis etiologies is mandatory to improve the management of the patients. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  18. Reproductive Health in Men and Women With Vasculitis

    Science.gov (United States)

    2014-06-25

    Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis

  19. Kidney Damage in Hemorrhagic Vasculitis Occurring in Childhood and Adulthood

    Directory of Open Access Journals (Sweden)

    O.V. Syniachenko

    2016-12-01

    of changes in interphalangeal joints of the feet and ligamentosis of knee joints. There is a direct connection between the renal pathology and lesion of maxillary joints and formation of tendovaginitis. Nephro­tic syndrome is diagnosed in 4 % of patients with HV, and chronic renal disease — in 28 % (the ratio of I, II, III and IV its stages was 10 : 5 : 1 : 1. The rate of progression of renal pathology has influenced by patients’ age at the onset of HV, the degree of di­sease activity, lesions of skin and gastrointestinal tract, the level of mean arterial pressure has been influenced by the involvement in the process of the pancreas, the peripheral vascular resistance — the liver, spleen and heart. Nephrotic syndrome and chronic kidney disease stage IV took place only in cases of onset of the disease in adulthood, hypertension was registered 1.6 times more likely, and decreased glomerular filtration rate — 2.4 times. IV and V morphological classes of HGN were found only in patients with the beginning of HV in adulthood, and VI — exceptionally in children. Morphology of the HGN in both groups resembled mesangioproliferative primary glomerulonephritis with tubulointerstitial component. In patients with disease onset in adulthood, lymphohistiocytic infiltration of blood vessels observed 2.3 times more frequently. Age at the onset glomerulonephritis affects the deposition in glomeruli IgM, and in vessels — IgA, IgG and IgM. And there are direct correlations between the age of the patients and glomerular deposition of IgA and vascular IgG. Conclusions. Regardless of the age of the patients, nephropathy occurs with the same frequency at the onset of HV, but in cases of onset of the di­sease in adulthood, the course of HGN is less favorable, followed by nephrotic syndrome, higher incidence of lesions of renal glo­meruli and stroma in the process of studying of kidney biopsies data, as well as different rates of progression of renal failure, levels of

  20. A Case Of Primary Central Nervous System Vasculitis Who Presented With Status Epilepticus

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    Sırma Geyik

    2014-12-01

    Full Text Available Primary central nervous system vasculitis (PCNV is limited with central nervous system and rare vasculitis that mostly seen in middle-aged men. PCNV vasculitis is usually presented that headache, dementia, stroke and multifocal common neurological symptoms. PCNV especially involves small medium-sized leptomeningeal and cortical arteries. 43 years old male patient who have been progressive forgetfulness and headache for 3 years. He applied with recurrent that before starting right focal and than sprawling whole body which generalized tonic-clonic seizures to us. During management that he was transfered to the intensive care unit due to status epilepticus (SE. Later than we found right hemiparesis, motor aphasia and right babinski positivity in neurologic examination. Diffusion restriction was revealed in left MCA territory in diffusion magnetic resonance imaging(MRI. EEG showed two types abnormality that a slow background ritm and epileptiform activity. Biochemistry of blood, complete blood count, blood sedimentation rate, CRP and markers of vasculitis were found in the normal range. Cerebral anjiography revealed that irregularities in the distal vascular areas and fusiform aneurysm at the top of basilar artery. He was consulted with rheumatology and diagnosed central nervous system vasculitis with the existing findings. Biopsy couldn't be taken from the brain to verify the diagnosis. Finally, we applied treatment that pulse steroid and cyclophosphamide to patient. This case has been presented due to emphasize that PCNV rarely may play a role in the etiology of recurrent stroke and status epilepticus.

  1. Plasmapheresis in a Patient With "Refractory" Urticarial Vasculitis

    OpenAIRE

    Kartal, Ozgur; Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman

    2012-01-01

    Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We su...

  2. Alzheimer's disease and CADASIL are heritable, adult-onset dementias that both involve damaged small blood vessels.

    Science.gov (United States)

    Marchesi, Vincent T

    2014-03-01

    This essay explores an alternative pathway to Alzheimer's dementia that focuses on damage to small blood vessels rather than late-stage toxic amyloid deposits as the primary pathogenic mechanism that leads to irreversible dementia. While the end-stage pathology of AD is well known, the pathogenic processes that lead to disease are often assumed to be due to toxic amyloid peptides that act on neurons, leading to neuronal dysfunction and eventually neuronal cell death. Speculations as to what initiates the pathogenic cascade have included toxic abeta peptide aggregates, oxidative damage, and inflammation, but none explain why neurons die. Recent high-resolution NMR studies of living patients show that lesions in white matter regions of the brain precede the appearance of amyloid deposits and are correlated with damaged small blood vessels. To appreciate the pathogenic potential of damaged small blood vessels in the brain, it is useful to consider the clinical course and the pathogenesis of CADASIL, a heritable arteriopathy that leads to damaged small blood vessels and irreversible dementia. CADASIL is strikingly similar to early onset AD in that it is caused by germ line mutations in NOTCH 3 that generate toxic protein aggregates similar to those attributed to mutant forms of the amyloid precursor protein and presenilin genes. Since NOTCH 3 mutants clearly damage small blood vessels of white matter regions of the brain that lead to dementia, we speculate that both forms of dementia may have a similar pathogenesis, which is to cause ischemic damage by blocking blood flow or by impeding the removal of toxic protein aggregates by retrograde vascular clearance mechanisms.

  3. Crescentic membranoproliferative glomerulonephritis and hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Enríquez, Ricardo; Sirvent, Ana Esther; Amorós, Francisco; Pérez, Miguel; Matarredona, Jaime; Reyes, Adolfo

    2005-01-01

    We describe the association of crescentic membranoproliferative glomerulonephritis and hypocomplementemic urticarial vasculitis syndrome. A 39-year-old woman presented edema and proteinuria and later a non-pruritic urticarial rash. Laboratory results showed nephrotic syndrome, hypocomplementemia and positive anti-C1q antibodies. Skin biopsy disclosed leukocytoclastic vasculitis. Acute renal failure developed. Renal biopsy revealed crescentic membranoproliferative glomerulonephritis. She was treated with corticosteroids and cyclosphosphamide with improvement of the renal function and partial remission of the nephrotic syndrome. Afterwards the nephrotic syndrome relapsed, mycophenolate mofetil in monotherapy was administered with reduction in proteinuria. As far as we know only 3 cases, 2 in children and one in an adult, of crescentic glomerulonephritis and hypocomplementemic urticarial vasculitis syndrome have been reported. In our patient renal manifestations preceded urticarial lesions. We provide information on the evolution during a 42-month follow-up.

  4. Severe tuberculous retinal vasculitis in healthy adults

    Directory of Open Access Journals (Sweden)

    Sin Yee Fang

    2015-09-01

    Full Text Available Ocular tuberculosis may be a presenting feature of tuberculous infection, especially with extensive occlusive retinal vasculitis among Asians. Being a curable disease, awareness of its presentation and high index of suspicion are paramount as prompt treatment can halt the disease progression and prevent visual loss. We presented three cases of ocular tuberculosis in young healthy adults who presented with progressive blurring of vision with florid retinal vasculitis seen on funduscopy. Two of them were with no bacillus Calmette-Guerin scar. Fundus fluorescein angiography confirmed the presence of occlusive vasculitis with extensive area of ischemia. All cases showed a raise in erythrocyte sedimentation rate and strongly positive Mantoux tests. Pan-retinal photocoagulation was given to all patients. Two cases responded well to anti-tubercular therapy followed by oral steroids and regained normal vision in both eyes. One patient was not started on anti-tubercular therapy as he requested to return to his native country for further treatment.

  5. Ischemic retinal vasculitis in an 18-year-old man with chickenpox infection

    Directory of Open Access Journals (Sweden)

    Poonyathalang A,Sukavatcharin S

    2014-02-01

    Full Text Available Anuchit Poonyathalang, Somsiri Sukavatcharin, Tharikarn Sujirakul Department of Ophthalmology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract: Ocular involvement after primary infection with varicella zoster virus is very rare. We report a case of a healthy 18-year-old man who presented with unilateral ischemic retinal vasculitis 10 days after the onset of chickenpox. He developed acute severe visual loss and a relative afferent pupillary defect in his right eye. Fundus imaging, optical coherence tomography, fundus fluorescence angiography, and electrophysiologic studies confirmed the diagnosis of retinal vasculitis, which led to generalized retinal ischemia. Although aggressive treatment with systemic steroids and antiviral drugs was administered, a poor visual outcome still resulted. Keywords: ischemic retinal vasculitis, chickenpox, varicella zoster virus

  6. Deferasirox-induced urticarial vasculitis in a patient with myelodysplastic syndrome.

    Science.gov (United States)

    Polat, Asude Kara; Belli, Asli Akin; Karakus, Volkan; Dere, Yelda

    2017-01-01

    Deferasirox is an iron chelator agent used in the treatment of diseases with iron overload, such as thalassemia and myelodysplastic syndrome. Although the majority of adverse reactions of deferasirox involve gastrointestinal symptoms and increase in serum creatinine and transaminases, skin rashes, such as maculopapular and urticarial eruptions, have also been reported. This study reports a case of myelodysplastic syndrome with urticarial vasculitis due to deferasirox therapy. Drug eruption was been confirmed by means of a challenge test, together with histopathological and clinical findings. To the best of our knowledge, we report the first case of deferasirox-induced urticarial vasculitis. Physicians should be aware of the possibility of urticarial vasculitis on deferasirox therapy and the fact that the discontinuation of the drug generally results in improvement.

  7. Down-Syndrome associated with MBL-deficiency, IgG-deficiency, vasculitis and mutated prothrombin.

    Science.gov (United States)

    Wolf, Hermann M; Stöllberger, Claudia; Finsterer, Josef

    2009-01-01

    The association of Down syndrome with mannose-binding lectin (MBL)-deficiency, recurrent infections and vasculitis has not been reported. We report a 30 year-old female with Down-syndrome associated with MBL-deficiency with the genotype LXA/HYD, IgG-deficiency, recurrent uro-genital infections, cutaneous vasculitis, G20.210A prothrombin mutation, deep venous thrombosis, and pulmonary embolism. MBL-deficiency in combination with IgG deficiency might have favored the development of recurrent uro-genital infections. Immunodeficiency might be also involved in the pathogenesis of cutaneous vasculitis. Deep venous thrombosis and pulmonary embolism were attributed to the genetically determined prothrombotic state and intake of oral contraceptives.

  8. Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression.

    Science.gov (United States)

    Chew, Gary Y J; Gatenby, Paul A

    2007-08-01

    We present a patient with previously diagnosed hypocomplementemic urticarial vasculitis syndrome, with skin, lung, and renal involvement, who presented with congestive cardiac failure. During the course of her hospitalization, she was also found to have profound proximal muscle weakness in both upper and lower limbs associated with raised creatinine kinase levels. A muscle biopsy was performed, which demonstrated evidence of an inflammatory myositis with vasculitis, which had returned despite on-going immunosuppression. This occurrence of a new autoimmune disease may well be an example of the "waste disposal" hypothesis.

  9. 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

  10. Granulomatosis with Polyangiitis Presenting with Coronary Artery and Pericardial Involvement

    Science.gov (United States)

    Dewan, Rohit; Trejo Bittar, Humberto E.; Lacomis, Joan; Ocak, Iclal

    2015-01-01

    Granulomatosis with polyangiitis is a systemic disease resulting in necrotizing vasculitis of small- and medium-sized vessels. Cardiac involvement is rare and when present usually manifests with pericarditis and coronary artery vasculitis. We report here a case of granulomatosis with polyangiitis involving the native coronary arteries, bypass graft, and pericardium with interesting imaging findings on contrast-enhanced CT and MRI. A 57-year-old man with a history of chronic headaches presented to the emergency room with syncope. Contrast-enhanced CT demonstrated extensive soft tissue attenuation around the native coronary arteries and bypass graft. Contrast-enhanced MRI demonstrated enhancing nodular soft tissue surrounding the coronary arteries, bypass graft, and pericardium. Pericardial biopsy revealed a necrotizing granulomatous pericarditis with vasculitis concerning for granulomatosis with polyangiitis. The patient demonstrated MPO-positive and PR-3 negative serologies. After being discharged on rituximab and prednisone, follow-up CT 3 years later showed significant improvement of the soft tissue thickening surrounding the coronary arteries, bypass graft, and pericardium. PMID:26798536

  11. Renal Survival in Proteinase 3 and Myeloperoxidase ANCA-Associated Systemic Vasculitis

    NARCIS (Netherlands)

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

    2013-01-01

    Background and objectivesThis study evaluated predictors for patient and renal survival in patients with ANCA-associated vasculitis (AAV) with and without renal involvement.Design, setting, participants, & measurementsThere were 273 consecutive AAV patients from January 1990 until December 2007 who

  12. Candida albicans-associated necrotizing vasculitis producing life-threatening gastrointestinal hemorrhage.

    LENUS (Irish Health Repository)

    Sargent, Jeremy

    2012-02-01

    Patients undergoing treatment of acute lymphoblastic leukemia are at risk for fungal infections including disseminated candidiasis. We describe a case of systemic Candida albicans infection associated with life-threatening gastrointestinal hemorrhage due to unusual necrotizing vasculitis involving the gastrointestinal tract. We explore the association between Candida and such vasculopathy.

  13. Urticarial Vasculitis in a Teenage Girl

    Science.gov (United States)

    Vaughan, Amy; Wolford, Juliet

    2013-01-01

    This case involves a 13-year-old female who presented to the pediatrician for a routine check-up with complaints of a long history of intermittent diarrhea followed by a severe rash lasting for up to a week afterwards. The mother had described her daughter’s condition to multiple physicians, several whom had seen her during flare-ups. The nonmigratory lesions resembled “hives” with a single lesion lasting for 48 to 72 hours and resolving into what her parent described as a bruise. They often diagnosed her daughter with urticaria and prescribed steroids, which did resolve the acute flare-ups. None of the physicians, however, focused on the disease’s evolution and chronicity in an effort toward diagnosis and prevention. The patient was referred by her pediatrician to a dermatologist who diagnosed the patient with urticarial vasculitis. She was initially started on dapsone 25 mg and was increased over a period of months to a maintenance dose of 100 mg daily. She has had no recurrences in her cutaneous or systemic symptoms on this dose. She is closely monitored by her dermatologist on a regular basis with twice yearly complete blood counts. Several attempts have been made to discontinue the dapsone, resulting in a flare of her gastrointestinal symptoms. This patient suffered with this condition for almost 10 years. This is a reminder that spending extra time to think through a patient’s problem early on may prevent years of suffering for patients and their families. PMID:26425571

  14. Urticarial Vasculitis in a Teenage Girl

    Directory of Open Access Journals (Sweden)

    Aaron McGuffin MD

    2013-04-01

    Full Text Available This case involves a 13-year-old female who presented to the pediatrician for a routine check-up with complaints of a long history of intermittent diarrhea followed by a severe rash lasting for up to a week afterwards. The mother had described her daughter’s condition to multiple physicians, several whom had seen her during flare-ups. The nonmigratory lesions resembled “hives” with a single lesion lasting for 48 to 72 hours and resolving into what her parent described as a bruise. They often diagnosed her daughter with urticaria and prescribed steroids, which did resolve the acute flare-ups. None of the physicians, however, focused on the disease’s evolution and chronicity in an effort toward diagnosis and prevention. The patient was referred by her pediatrician to a dermatologist who diagnosed the patient with urticarial vasculitis. She was initially started on dapsone 25 mg and was increased over a period of months to a maintenance dose of 100 mg daily. She has had no recurrences in her cutaneous or systemic symptoms on this dose. She is closely monitored by her dermatologist on a regular basis with twice yearly complete blood counts. Several attempts have been made to discontinue the dapsone, resulting in a flare of her gastrointestinal symptoms. This patient suffered with this condition for almost 10 years. This is a reminder that spending extra time to think through a patient’s problem early on may prevent years of suffering for patients and their families.

  15. Plasmapheresis in a patient with "refractory" urticarial vasculitis.

    Science.gov (United States)

    Kartal, Ozgur; Gulec, Mustafa; Caliskaner, Zafer; Nevruz, Oral; Cetin, Turker; Sener, Osman

    2012-07-01

    Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We suggest that plasmapheresis be considered as an option in patients with severe or treatment-resistant urticarial vasculitis.

  16. [Agranulocytosis and vasculitis in a cocaine addict: levamisole, the hidden culprit].

    Science.gov (United States)

    Lemaignen, A; Goulenok, T; Kalamarides, S; Plat, A; Pfau, G; Fantin, B

    2014-10-01

    Adulterants are compounds added to street drugs to increase profits for the seller. Levamisole, a veterinary antihelminthic agent, has become the most common adulterant of cocaine. The prevalence of levamisole in samples of cocaine is increasing. Levamisole can lead to neutropenia and to a dramatic vasculopathy and even vasculitis of small and medium-size blood vessels. We here reported the first French case of levamisole related toxicity, due to cocaine use in a 50-year-old man, revealed by fever and agranulocytosis, high titters of antineutrophil cytoplasmic antibodies (ANCA), anticoagulant and positive Coombs tests. Outcome was slowly favorable with exposition withdrawal. Clinicians should be aware that agranulocytosis or vasculitis or vasculopathy could be related to levamisole toxicity in individuals who use cocaine. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  17. 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.

  18. Autoimmunity in chronic urticaria and urticarial vasculitis.

    Science.gov (United States)

    Napoli, D C; Freeman, T M

    2001-07-01

    In contrast to acute urticaria, etiology cannot be identified in most cases of chronic urticaria. Recent evidence suggests that a subset of patients with chronic urticaria may have an autoimmune basis for their condition. The demonstration of antithyroid autoantibodies in some patients with chronic idiopathic urticaria (CIU) provides support for an association. However, the discovery of a positive skin test response to intradermal injection of autologous serum in as many as 60% of patients with CIU led to the identification of autoantibodies to IgE and the alpha-chain of the high-affinity IgE receptor, Fc epsilon RI alpha. Additional studies have demonstrated that some of these autoantibodies are capable of releasing histamine from donor basophils and mast cells. This article reviews the literature that addresses a possible autoimmune etiology in a subset of patients with CIU. Urticarial vasculitis is differentiated from chronic urticaria based on clinical features and biopsy findings of leukocytoclastic vasculitis. Most cases of urticarial vasculitis are secondary to an underlying systemic disease. The presence of autoantibodies has also been demonstrated in a subset of patients with primary urticarial vasculitis. This article briefly reviews some of this data.

  19. Relapsing granulomatosis with polyangiitis with severe lung and upper respiratory tract involvement successfully treated with rituximab.

    Science.gov (United States)

    Masiak, Anna; Zdrojewski, Zbigniew

    2017-01-01

    Granulomatosis with polyangiitis (GPA) is a chronic, relapsing, primary systemic small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies. The disease is characterised by the involvement of various organs. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. The combination of glucocorticoids and cyclophosphamide remains the standard therapy for patients with generalised GPA; nevertheless, some patients do not respond satisfactorily to this treatment. According to EULAR-EDTA recommendations for the management of ANCA-associated vasculitis, RTX should be considered for remission-induction of new-onset as well as major relapse of organ-threatening or life-threatening GPA. Here we present a 35-year-old patient with relapsing GPA successfully treated with RTX. We also highlight the infectious complications of immunosuppressive treatment.

  20. Henoch-Schönlein purpura without systemic involvement beginning with acute scrotum and mimicking torsion of testis

    Directory of Open Access Journals (Sweden)

    Levent Verim

    2013-04-01

    Full Text Available Henoch-Schönlein purpura (HSP is the most common systemic vasculitis in children. Typical presentations of HSP are palpable purpura of the small vessels in the hips and lower limbs, abdominal pain, arthritis, and hematuria. Scrotal involvement manifested by the presence of scrotal pain and swelling during the course of the disease is rarely seen. HSP without systemic involvement with acute scrotum mimicking testicular torsion is even rare in the medical literature. In most cases, patients with this disease achieve complete recovery. Herein, we report an interesting HSP case with skin symptoms but without systemic involvement and then progression to acute scrotum resembling torsion of testis.

  1. Vessel Operating Units (Vessels)

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — This data set contains data for vessels that are greater than five net tons and have a current US Coast Guard documentation number. Beginning in1979, the NMFS...

  2. Therapeutic advances in the treatment of vasculitis

    OpenAIRE

    Eleftheriou, D.; P.A. Brogan

    2016-01-01

    Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trial...

  3. Warfarin-induced leukocytoclastic vasculitis and proteinuria

    Directory of Open Access Journals (Sweden)

    Khalid Jumean

    2016-01-01

    Full Text Available Warfarin is typically prescribed for patients with thromboembolic diseases and atrial fibrillation. In addition to the complications of bleeding, allergic skin reaction is one of its rare adverse effects. We herein report a case of a 79 year old male patient with leukocytoclastic vasculitis and proteinuria secondary to warfarin. The warfarin was discontinued and oral prednisone therapy was initiated. The cutaneous lesions and the proteinuria resolved thereafter.

  4. What does the future hold for clinical studies in vasculitis?

    Science.gov (United States)

    Hoffman, G S

    2011-05-01

    The era prior to 1990 was a time of careful observation of disease presentation, course, outcomes and meticulous pathology studies. These mainly single-centre studies introduced new life-saving therapies for drugs still used effectively today. In the 1970-1980s, cyclophosphamide (CyP) added to glucocorticosteroids (GCS) was shown to be life-saving. The trade-off was often severe adverse events. Some forms of vasculitis were found not as ominous as thought initially. Some could be treated with safer drugs [e.g. methotrexate (MTX)]. However, whether mild or severe, patients were not cured. From 1990 to the present large collaborative networks have provided studies were not possible heretofore. Randomized controlled trials captured and manipulated vast amounts of data, banked biological specimens and shared these resources and intellectual capital, moving the field forward at an extraordinary pace. We now know that even for severe forms of granulomatosis and polyangiitis [granulomatosis with polyangiitis (GPA), Wegener's granulomatosus (WG)], microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS), we do not need to use CyP for extended periods. We have learned recently that rituximab is as effective as CyP for severe WG and MPA. We should never again see the permanent toxicities born from years of chronic CyP use. However, short courses of CyP remain useful and can be life-saving. Step-down therapy from CyP is now a standard of care, perhaps to be replaced by rituximab in the future. If one accepts the premise that there are few cures at present for idiopathic large- and small-vessel vasculitis, we will serve our patients well if we can determine the most effective initial therapy that leads to a maintenance strategy for remission with least risk. Ultimately, we wish to identify causes of vasculitis so they can be used as a wedge to secure cures. Unmet needs and strategies are as follows: (1) to increase the numbers of vasculitis-trained physicians; (2) to

  5. [Spontaneous achilles tendon rupture in granulomatous vasculitis].

    Science.gov (United States)

    Benthien, Jan Philipp; Delling, G; Rüther, W

    2003-08-01

    A 66-year old patient sustained a non-traumatic rupture of her left achilles tendon. She suffered from Sjögren's syndrome which occurred in conjunction with a systemic vasculitis, and recurrent episcleritis. The combination of Sjögren's syndrome and systemic vasculitis is well known. Subsequently, she was treated with high-dose systemic steroids over a period of 2 years. In order to reduce the amount of steroids due to preexisting severe osteoporosis and thoracic vertebral fractures, her medication was changed to cyclophosphamide shortly before her injury. Intraoperatively, a granuloma was discovered at the site of the rupture. This granuloma had infiltrated most of the achilles tendon at this site and virtually replaced viable tendon tissue. Originally, the rupture was supposedly due to the high dose steroids. This theory had to be revised according to the intraoperative findings. Following excision of the granuloma and operative treatment of the achilles tendon rupture, the continuity of the tendon could be completely restored. A MRI scan 3 months after the procedure demonstrated a completely healed Achilles tendon. Spontaneous achilles tendon rupture due to a granuloma in patients with vasculitis seems to be a rare event. However, tendon ruptures in combination with systemic lupus erythematodes have been described. Mostly, these events are attributed to long term application of steroids. Spontaneous rupture in combination with high dose treatment of steroids seems to be an underestimated problem.

  6. A classic mimicker of systemic vasculitis.

    Science.gov (United States)

    Moreno-Ariño, Marc; Ortiz-Santamaria, Vera; Deudero Infante, Aída; Ayats Delgado, Montserrat; Novell Teixidó, Francesc

    2016-01-01

    Embolic and constitutional manifestations of intracavitary cardiac tumors are included within the classic mimickers of systemic vasculitis, especially in those in which there are no cardiac manifestations. We present a case report of atrial myxoma in which the patient only presented systemic symptoms and in whom an initial diagnostic approach of systemic vasculitis was made. We also performed a literature search of the cases described. A case report of atrial myxoma with atypical presentation manifested as a systemic disease with no concomitant cardiac symptoms is described. The case report is discussed and 11 cases of atrial myxoma pseudovasculitis described in the literature are reviewed, emphasizing their similarities and differences. Constitutional symptoms and cutaneous manifestations were the most common. Most of the cases showed partial response to glucococorticosteroid treatment, reinforcing the theory of the inflammatory role in its pathogenesis. Mean delayed time to diagnosis was 12.27 months. Atrial myxoma is a systemic vasculitis mimicker, this being difficult to diagnose in the absence of cardiac manifestations. This delay in diagnosis entails serious complications. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  7. Levamisole-Induced Leukocytoclastic Vasculitis with Negative Serology in a Cocaine User.

    Science.gov (United States)

    Salehi, Mashal; Morgan, Michael P; Gabriel, Abigail

    2017-06-08

    BACKGROUND Levamisole is a common adulterant of cocaine. It can cause agranulocytosis and cutaneous vasculitis that can possibly lead to cutaneous necrosis. In all reported cases of levamisole-induced vasculitis, it has been described as a clinical syndrome characterized by a constellation of typical clinical features and a positive serum serology for ANCA levels, especially very high-titer p-ANCA levels, in the background of cocaine abuse. However, patients may have a negative serology and here, we present the first such case. CASE REPORT A 58-year-old African American man with a history of polysubstance abuse, 4 days after last cocaine use, presented with sudden onset of painful pruritic rash and polyarthralgias. He was found to have normal vital signs, with bilateral tender knees and erythematous-purplish maculopapular lesions involving the abdomen and the left thigh. Laboratory work-up was significant for elevated CRP, negative c-ANCA, p-ANCA ANA, and RA levels, and a positive urine toxicology for cocaine. Urine analysis by high-performance liquid chromatography was positive for levamisole. Ultimately, a final diagnosis was made by skin biopsy, which revealed findings suggestive of leukocytoclastic vasculitis. CONCLUSIONS Cutaneous leukocytoclastic vasculitis can be caused by levamisole, which is used as an adulterant in cocaine. Most cases are associated with positive ANCA levels; however, a negative serology is also a possibility.

  8. Hypocomplementemic urticarial vasculitis in mixed connective tissue disease.

    Science.gov (United States)

    Calistru, Ana Maria; Lisboa, Carmen; Cruz, Maria João; Delgado, Luis; Poças, Licínio; Azevedo, Filomena

    2010-12-15

    Urticarial vasculitis is characterized clinically by urticaria-like skin lesions and histologically by leukocytoclastic vasculitis. It may be idiopathic or associated with various conditions such as infections, hematologic disorders, drugs, and connective tissue diseases, primarily systemic lupus erythematosus; an association with mixed connective tissue disease (MCTD) has rarely been reported. We present a case of hypocomplementemic urticarial vasculitis in a patient with MCTD that responded to hydroxychloroquine after a period of corticosteroid dependence.

  9. Treatment with intravenous gammaglobulin in pediatric patients with primary vasculitis

    Directory of Open Access Journals (Sweden)

    Camacho Meza Ignacio

    2014-07-01

    Full Text Available Nowadays intravenous immunoglobulins have been used in differ- ent vasculitis with various results for each condition, being more recommended for its use in Kawasaki disease and ANCA-associated vasculitis. However, there is still no solid evidence to support its use in the entire group of these diseases. We present a review of the existing literature related to the use of intravenous immunoglobulin for the treatment of primary vasculitis in childhood.

  10. Biomarkers of IgA vasculitis nephritis in children

    Science.gov (United States)

    Pillebout, Evangeline; Jamin, Agnès; Ayari, Hamza; Housset, Pierre; Pierre, Melissa; Sauvaget, Virginia; Viglietti, Denis; Deschenes, Georges

    2017-01-01

    Henoch–Schönlein purpura is a systemic vasculitis characterized by IgA deposits, which target the skin, joints, and kidneys, among other organs. In children, prognosis is often good but little is known about biomarkers of pediatric nephritis. We hypothesized that biological markers, including cytokines, immunoglobulins, IgA-immune complexes, IgA glycosylation and neutrophil gelatinase-associated lipocalin (NGAL), may discriminate IgA vasculitis (IgAV) pediatric patients with renal involvement from those without renal involvement. Fifty children at the time of IgAV rash between 2010 and 2015 were prospectively enrolled and compared to 21 controls. All patients were assessed for clinical and biological parameters at the time of diagnosis, including the levels of cytokines, immunoglobulins, immune complexes, IgA glycosylation and NGAL in serum and urine. Among IgAV patients, 33 patients exhibited nephritis (IgAV-N) and 17 children were without nephritis (IgAV-woN). The serum level of galactose-deficient (Gd)-IgA1 (pmarkers, urinary IgA and IgM had the highest AUC (0.86 and 0.87 respectively, p<0.0001). This prospective cohort study furthers our understanding of the pathophysiology of IgAV. We identified biomarkers that are able to distinguish patients initially with or without nephritis. To conclude, serum Gd-IgA1 and urinary IgA, IgG, IgM, IL-6, IL-8, IL-10, and IgA-IgG and IgA-sCD89 complexes could identify IgAV pediatric patients with renal involvement at the time of diagnosis. PMID:29190714

  11. Vasculitis Terms A to Z

    Science.gov (United States)

    ... may be mimickers of tuberculosis. MRI Magnetic Resonance Imaging: Another fancy x-ray, similar to a CT scan. MRI scans also provide cross-sectional images of body organs. Because MRI technology involves the use of a large magnet, people ...

  12. 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.

  13. Pure Motor Aphasia Developed After Cerebral Vasculitis Due To Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Murat Terzi

    2011-09-01

    Full Text Available Systemic lupus erythematosus (SLE is a complex multisystem disease that may involve the central and peripheral nervous systems. Common clinical findings include seizures, depression, psychosis, aseptic meningitis, headache, cranial and peripheral neuropathies. The incidence of stroke as a neurological involvement in patients with SLE is between 3-20%. Possible reasons for the development of stroke are suggested as hypercoagulability and thrombosis due to antiphospholipid antibodies, cerebral vasculitis, cerebral embolus due to Libman Sacks endocarditis and hypertension due to the disease itself or long term corticosteroid use. We present clinical, laboratory and radiological imaging findings that confirm cerebral vasculitis of a SLE case with the clinical presentation of acute stroke in this study.

  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. Paraneoplastic vasculitis associated to pelvic chondrosarcoma: a case report

    Directory of Open Access Journals (Sweden)

    Arvinius Camilla

    2016-01-01

    Full Text Available Vasculopathic syndromes have been associated with hematological and solid organ malignancies. The pathogenesis of these syndromes remains largely unknown and there are no biologic markers identified. Whether it is or is not a paraneoplastic syndrome is under discussion, the close temporal relationship of cancer and vasculitis suggests that these vasculitides are a paraneoplastic condition. We report a case of a 45-year-old female patient with pelvic chondrosarcoma who underwent surgical treatment and started to present visual loss, systemic inflammatory response syndrome (SRIS, cardiac insufficiency, hepatosplenomegaly, cholestasis as well as pulmonary bleeding suggesting a sarcoma-associated vasculitis. All antibodies were negative as in secondary vasculitis. After corticoideal therapy the vasculitis resolved and at 3-year follow-up the patient had not showed any further medical complications or recurrences of the vasculitis. The parallel evolution of the vasculitis and the solid tumor combined with the resolution of the vasculitis after corticotherapy enhances the likelihood of a paraneoplastic vasculitis associated with a chondrosarcoma according to literature review.

  16. Cutaneous vasculitis as a presenting manifestation of acute myeloid leukemia.

    Science.gov (United States)

    Jayachandran, Nambiar Veettil; Thomas, Joe; Chandrasekhara, Pradeep Kumar Shenoy; Kanchinadham, Suresh; Kadel, Jugal Kishore; Narsimulu, Gumdal

    2009-04-01

    One of the rare causes of secondary vasculitides is malignancy. Hematological malignancies produce secondary vasculitis more frequently than solid malignancies. Here in we report a case of acute myeloid leukemia presenting with anti-neutrophil cytoplasmic antibody-positive vasculitis. This case highlights the importance of looking for underlying malignancies, especially leukemias in patients presenting with features of systemic vasculitides.

  17. Persistent cryoglobulinemic vasculitis following successful treatment of hepatitis C virus.

    Science.gov (United States)

    Levine, James W; Gota, Carmen; Fessler, Barri J; Calabrese, Leonard H; Cooper, Sheldon M

    2005-06-01

    There is a well established link between type II mixed cryoglobulinemia (MC) and hepatitis C virus (HCV) infection, and HCV is believed to be the cause of cryoprotein formation and tissue deposition. Successful treatment of HCV infection has resulted in resolution of cryoglobulinemia and vasculitis. We describe 4 patients who had persistent MC and vasculitis despite successful eradication of HCV with antiviral therapy.

  18. Relationship between pressure and digital vasculitis in rheumatoid disease.

    OpenAIRE

    Edwards, J C

    1980-01-01

    The sites of 92 rheumatoid nailfold lesions were compared with sites of pressure as indicated by tissue blanching during gripping. A close correspondence between sites of blanching and sites of vasculitis was found, and this is discussed in relation to the possible role of vascular compression and blood stasis in the development of rheumatoid vasculitis.

  19. Retinal Vasculitis in Anti-Synthetase Syndrome.

    Science.gov (United States)

    Donovan, Christopher P; Pecen, Paula E; Baynes, Kimberly; Ehlers, Justis P; Srivastava, Sunil K

    2016-09-01

    A 31-year-old woman with a history of anti-synthetase syndrome-related myositis and interstitial lung disease presented with acute-onset blurry vision and rash on her hands and feet. Visual acuity was hand motion in her right eye and 20/40 in her left eye. Dilated fundus exam showed extensive retinal vasculitis, diffuse intraretinal hemorrhages, and subretinal fluid. Optical coherence tomography revealed significant macular thickening, and fluorescein angiography revealed vascular leakage with peripheral nonperfusion. Aggressive systemic immunosuppression was initiated, with gradual resolution of her disease during 8 months of follow-up. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:874-879.]. Copyright 2016, SLACK Incorporated.

  20. Leukocytoclastic Vasculitis as a Complication of Recombinant Granulocyte Colony-Stimulating Factor Therapy in a Heart Transplant Patient

    Directory of Open Access Journals (Sweden)

    Giovanbattista Ippoliti

    2014-01-01

    Full Text Available Recombinant granulocyte colony-stimulating factor (rG-CSF is a myeloid growth factor that is widely used in haematology to recover neutropenia secondary to myelosuppressive chemotherapy. Leukocytoclastic vasculitis is an acknowledged side effect of the above therapy. Its pathogenesis involves many mechanisms that collectively induce an increase in neutrophil function and a subsequent release of cytokines. Here, we report a case of leukocytoclastic vasculitis proven by skin biopsy, following the use of rG-CSF in a heart transplant patient with leukopenia secondary to immunosuppressive therapy.

  1. [A rapidly progressive orbital tumor in a seven-year-old girl revealing a primitive systemic vasculitis].

    Science.gov (United States)

    Picard, Cécile; Borgnat, Florent; Chastang, Marie-Caroline; Fleury, Aude; Guibaud, Laurent; Dijoud, Frédérique

    2014-12-01

    Orbital tumor lesions in pediatric population encompass a wide range of pathological processes, which are very different in etiology and prognosis. They usually require an emergency in histological diagnosis because of their quick growth. Beside malignant and benign tumors, the inflammatory pseudo-tumors group included dysimmune orbital involvement secondary to systemic vasculitis such as granulomatosis with polyangiitis (ex-Wegener's granulomatosis). We report the case of a seven-year-old girl suffering from a severe primitive ANCA vasculitis, revealed by an orbital mass. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  2. Urticarial vasculitis appearing in the progression of systemic sclerosis.

    Science.gov (United States)

    Kato, Yoko; Aoki, Mikako; Kawana, Seiji

    2006-11-01

    We report a case of urticarial vasculitis that appeared during the course of limited cutaneous systemic sclerosis. The urticarial lesions responded to systemic administration of prednisolone. After the appearance of urticarial vasculitis, the progression of scleroderma in distal sites of her elbow and knee joint became apparent. We consider this case to be consistent with limited cutaneous systemic sclerosis. The patient started treatment with prednisolone and her edema as well as scleroderma softened gradually. We analyzed, by immunohistochemistry, the number of tryptase-positive mast cells of this case in the lesions of urticarial vasculitis as well as systemic sclerosis. The number of tryptase-positive mast cells in the lesions of urticarial vasculitis as well as systemic sclerosis was significantly increased compared to normal skin (P urticarial vasculitis and systemic sclerosis as a common factor.

  3. Cryoglobulinemic vasculitis in a patient with CREST syndrome.

    Science.gov (United States)

    Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D

    2014-10-01

    Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. Copyright © 2014 Elsevier Ltd. All rights reserved.

  4. Eyelid Necrosis and Secondary Cicatrical Ectropion Secondary to Levamisole-Associated Vasculitis.

    Science.gov (United States)

    Ramesh, Sathyadeepak; Sobti, Deepak; Mancini, Ronald

    A 56-year-old female presented with eyelid necrosis secondary to systemic levamisole-induced vasculitis. Skin biopsy revealed necrotic epidermis with small-vessel thrombosis, fibrinoid reaction, and neutrophilic infiltration of vessel walls in the dermis with +pANCA. She was treated with plasmapheresis and steroids. Six months later, she developed severe, symptomatic cicatrical ectropion with marked anterior lamellar shortage and middle lamellar contracture. Scar release in the middle lamellar plane with lateral tarsal strip procedures was performed, with full-thickness skin grafts from the upper eyelids. She remained fully epithelialized postoperatively with improvement in symptoms, although she incomplete graft take due to her eyelid necrosis and compromised dermal blood supply.

  5. Pressure vessel design manual

    CERN Document Server

    Moss, Dennis R

    2013-01-01

    Pressure vessels are closed containers designed to hold gases or liquids at a pressure substantially different from the ambient pressure. They have a variety of applications in industry, including in oil refineries, nuclear reactors, vehicle airbrake reservoirs, and more. The pressure differential with such vessels is dangerous, and due to the risk of accident and fatality around their use, the design, manufacture, operation and inspection of pressure vessels is regulated by engineering authorities and guided by legal codes and standards. Pressure Vessel Design Manual is a solutions-focused guide to the many problems and technical challenges involved in the design of pressure vessels to match stringent standards and codes. It brings together otherwise scattered information and explanations into one easy-to-use resource to minimize research and take readers from problem to solution in the most direct manner possible. * Covers almost all problems that a working pressure vessel designer can expect to face, with ...

  6. A Case of Rituximab Use as an Induction and Maintenance of Remission in ANCA-Associated Vasculitis

    Directory of Open Access Journals (Sweden)

    Neveen Awad

    2016-01-01

    Full Text Available Antineutrophil cytoplasmic antibody- (ANCA- associated vasculitis (AAV is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA- associated vasculitis (AAV was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials.

  7. ANCA Associated Vasculitis Secondary to Levamisole-Adultered Cocaine with Associated Membranous Nephropathy: A Case Series.

    Science.gov (United States)

    Collister, David; Sathianathan, Christie; Ryz, Krista; Karpinski, Martin; Bernstein, Keevin; Gibson, Ian W

    2017-01-01

    Cocaine is a risk factor for acute kidney injury and chronic kidney disease with progression to end-stage renal disease. Levamisole is an adulterant that is added to cocaine to enhance its euphoric effects. Levamisole-adulterated cocaine (LAC) is associated with the distinct clinical syndromes of agranulocytosis, leukocytoclastic vasculitis, cocaine-induced midline destructive lesions (CIMDL), and ANCA-associated vasculitis (AAV) with pauci-immune necrotizing glomerulonephritis. We reviewed all cases of AAV secondary to LAC at our institution. We report 3 cases of AAV secondary to LAC and associated membranous nephropathy (MN). The first and second cases are concurrent AAV secondary to LAC and associated MN while the third case involves the development of MN after AAV secondary to LAC. Clinicians should be aware of this novel association of LAC with MN. © 2017 S. Karger AG, Basel.

  8. p-ANCA-Associated Vasculitis Caused by Levamisole-Adulterated Cocaine: A Case Report

    Directory of Open Access Journals (Sweden)

    Michelle R. Carter

    2013-01-01

    Full Text Available A rare case of a patient with an unusual skin rash after using cocaine is presented. A clinical diagnosis of levamisole-induced vasculitis was made based on initial presentation of purpuric skin lesions involving the ears and positive cocaine on urine toxicology screening. The diagnosis was confirmed after laboratory findings of neutropenia, positive serum levamisole, and a histopathologic test of the skin lesions. The pathophysiology of this condition is discussed. Treatment with methylprednisolone and prednisone led to the resolution of the neutropenia and complete recovery of the skin lesions. With the growing use of levamisole-tainted cocaine, clinicians should be aware of the symptoms of vasculitis and neutropenia induced by this combination of drugs to avoid unnecessary tests and delayed diagnosis.

  9. The role of metals in autoimmune vasculitis: epidemiological and pathogenic study.

    Science.gov (United States)

    Stratta, P; Messuerotti, A; Canavese, C; Coen, M; Luccoli, L; Bussolati, B; Giorda, L; Malavenda, P; Cacciabue, M; Bugiani, M; Bo, M; Ventura, M; Camussi, G; Fubini, B

    2001-04-10

    A possible relationship between Silica (Si) exposure and antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis has been reported. Furthermore, tuberculosis (TBC) has been frequently described in patients with silicosis, and TBC infection shares with ANCA-associated vasculitis the formation of granulomas. Therefore, an intriguing network including Silica, Vasculitis, TBC and ANCA might be hypothesized. The aim of this work was to further investigate these correlations using both epidemiological and pathogenic approaches. Study I--epidemiological study. A case-control study to compare the occupational histories of 31 cases of biopsy proven vasculitis (18 pauci-immune crescentic glomerulonephritis, 9 microscopic polyangitis, 4 Wegener's granulomatosis) with those of 58 age, sex and residence-matched controls (affected by other kidney diseases), was performed. Occupational Health physicians designed an appropriate questionnaire in order to evaluate a wide spread of exposures and calculate their entity by the product of Intensity x Frequency x Duration. Study II--tuberculosis association. A case-control study to evaluate the frequency of a previous history of tuberculosis (TBC) in 45 patients with vasculitis and 45 controls were performed. Study III--ANCA positivity. A case-control study to evaluate the presence of ANCA was performed by testing blood samples of 64 people with previous professional exposure and 65 sex/age matched patients hospitalized in a General Medicine Unit. Furthermore, the same evaluation was made in a pilot study in 16 patients with ongoing or previous TBC. Study IV--experimental study. The oxygen free radicals (OFR) and IL-12 production (both involved in the pathogenesis of vasculitis) from human phagocytic cells stimulated with an amorphous (diatomaceous earth) and a crystalline (quartz) form of Si at the doses of 10 and 100 microg ml(-1) was evaluated. Study I--a positive history of exposure to Si resulted in significantly more

  10. Circumscribed cicatricial alopecia due to localized sarcoidal granulomas and single-organ granulomatous arteritis: a case report and systematic review of sarcoidal vasculitis.

    Science.gov (United States)

    Yazdani Abyaneh, Mohammad-Ali; Raghu, Preethi; Kircher, Kenneth; Kutzner, Heinz; Kortz, Alison; Carlson, John Andrew

    2015-10-01

    Vasculitis associated with sarcoid granulomas is an uncommon phenomenon. A 72-year-old female presented with an expanding region of circumscribed alopecia and scalp atrophy of 2 months duration. Biopsy showed non-caseating granulomas, dermal thinning, loss of follicles, fibrosis and muscular vessels disrupted by mixed lymphocyte, macrophage and giant-cell infiltrates. Affected vessels had loss and fragmentation of the elastic lamina, fibrous replacement of their walls and luminal stenosis (endarteritis obliterans). Dermal and vascular advential intralymphatic granulomas and lymphangiectases were found by D2-40 expression, suggesting lymphatic obstruction and poor antigen clearance. No evidence of a post-zoster eruption, systemic sarcoidosis or systemic giant-cell arteritis was found. Two years later, prednisone had halted - but not reversed - progression of her alopecia. Review of the literature showed two types of vasculitis associated with sarcoid granulomas: (i) acute, self-limited leukocytoclastic vasculitis and (ii) chronic granulomatous vasculitis (GV). Persistence of non-degradable material or antigen contributes to the pathogenesis of granulomatous inflammation. In this case, lymphatic obstruction probably impeded clearance of nonimmunologic and/or immunologic stimuli permitting and sustaining the development of sarcoid granulomas and sarcoid GV, ultimately causing scarring alopecia and cutaneous atrophy. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  11. Brief Report: Childhood-Onset Systemic Necrotizing Vasculitides: Long-Term Data From the French Vasculitis Study Group Registry.

    Science.gov (United States)

    Iudici, Michele; Puéchal, Xavier; Pagnoux, Christian; Quartier, Pierre; Agard, Christian; Aouba, Achille; Büchler, Matthias; Cevallos, Ramiro; Cohen, Pascal; de Moreuil, Claire; Guilpain, Philippe; Le Quellec, Alain; Roblot, Pascal; Serratrice, Jacques; Bachmeyer, Claude; Daugas, Éric; Terrier, Benjamin; Mouthon, Luc; Guillevin, Loïc

    2015-07-01

    To describe the initial features and long-term outcomes of childhood-onset small vessel and medium vessel systemic necrotizing vasculitides (SNVs), including antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) and polyarteritis nodosa (PAN). Data on patients with childhood-onset SNV registered in the French Vasculitis Study Group database were reviewed for demographic characteristics, clinical, laboratory, and histologic features, and outcomes. Disease activity was assessed with the Birmingham Vasculitis Activity Score and the Paediatric Vasculitis Activity Score, and damage was scored using the Vasculitis Damage Index. Relapse and survival rates and causes of death were analyzed. Fifty-six patients (35 with AAV and 21 with PAN) (median age at database enrollment 14 years [range 2-17]) were included in the study. The median duration of followup was 96 months (range 1-336); two-thirds of the patients were followed up beyond 18 years of age. Six patients (11%) died, mostly of SNV-related causes. Relapse rates ranged from 33% for microscopic polyangiitis to 50% for eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and 83% for granulomatosis with polyangiitis (Wegener's), with similar rates among AAV and PAN patients (76% and 75%, respectively); neither overall survival nor relapse-free survival differed significantly between the 2 disease groups. Rates of relapse increased after 18 years of age, both among patients with AAV and among patients with PAN. At the last followup evaluation, AAV patients had more major flares and more severe accrued damage compared with PAN patients. Despite similar relapse rates, patients with childhood-onset AAVs experienced more major flares with more cumulative damage than those with pediatric PAN. Treatments aimed at reducing the rates of mortality and relapse in this patient group need to be developed and assessed. © 2015, American College of Rheumatology.

  12. Clinical features and etiology of retinal vasculitis in Northern Thailand

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    Supanut Apinyawasisuk

    2013-01-01

    Full Text Available Purpose: To report on the clinical features and etiology of patients with retinal vasculitis (RV. Materials and Methods: We reviewed medical records of 47 patients (75 affected eyes diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered. Results: Etiology of RV included infectious causes in 10/47, (21% while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%. Eales′ disease and Behcet′s disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60% among those with infectious disorders. RV was bilateral in 28/47 (60% patients. Retinal veins were most commonly affected (72%, 34/47. Involvement of arteries was present in 12/47 (25% and was associated with viral infections and Behcet′s disease. Ocular complications developed in 60/75 (80% eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%. Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%. Conclusions: RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet′s disease. Tuberculosis was the most common infectious cause.

  13. Retinal vasculitis in a patient with abdominal tuberculosis

    Science.gov (United States)

    Kannan, Balaji; Govindarajan, Kummararaj; Kummararaj, Sherin; Balaji, Vijayalakshmi; Natarajapillai, Venugopal

    2010-01-01

    Tuberculosis (TB) is one of the most common systemic diseases in India. Intraocular TB is however, rare. Retinal vasculitis is a relatively rare manifestion of intraocular TB. We report a case of bilateral retinal vasculitis in a 19-year-old girl with abdominal tuberculosis. The patient responded well to anti-TB treatment along with a short course of low dose oral steroids. Vision in her right eye however remained compromised due to residual maculopathy. This is the first report of bilateral retinal vasculitis due to colonic TB. PMID:21217901

  14. Lepra reaction with lucio phenomenon mimicking cutaneous vasculitis.

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    Misra, Durga Prasanna; Parida, Jyoti Ranjan; Chowdhury, Abhra Chandra; Pani, Krushna Chandra; Kumari, Niraj; Krishnani, Narendra; Agarwal, Vikas

    2014-01-01

    Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis.

  15. Lepra Reaction with Lucio Phenomenon Mimicking Cutaneous Vasculitis

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    Durga Prasanna Misra

    2014-01-01

    Full Text Available Leprosy is a disease typically found in the tropics. Patients with leprosy can have varying presentation with constitutional symptoms, joint pains, skin nodules, and rarely a vasculitis-like picture with skin ulcers and neuropathy. We present a young lady who presented with the rare manifestation of skin infarcts mimicking cutaneous vasculitis, diagnosed on histopathology to have Lucio phenomenon on a background of lepromatous leprosy. With increasing migration and widespread use of biologic response modifiers, clinicians all over the world need to be aware of various presentations of leprosy as well as needing to keep an open mind while considering the differential diagnoses of vasculitis.

  16. Vasculitis inducida por metimazol: Reporte de caso

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    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.

  17. Hypocomplementemic urticarial vasculitis arising in a patient with immunoglobulin G4-related disease.

    Science.gov (United States)

    Takao, Mariko; Hamada, Toshihisa; Kaji, Tatsuya; Ikeda-Mizuno, Kazuko; Takehara-Yasuhara, Chinatsu; Ichimura, Kouichi; Yanai, Hiroyuki; Yshino, Tadashi; Iwatsuki, Keiji

    2016-04-01

    Hypocomplementemic urticarial vasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease is a lymphoproliferative disorder characterized by sclerosing pancreatitis, retroperitoneal fibrosis, sclerotic cholangitis, acute interstitial nephritis, and Mikulicz's disease, and associated with elevated levels of IgG4 and hypocomplementemia. Various cutaneous lesions may occur in IgG4-related disease. We report a 74-year-old woman with HUV, followed by systemic organ involvement suggestive of IgG4-related disease. Symptoms included submandibular gland swelling, lymphadenopathy, interstitial nephritis, and a thyroid nodule. Laboratory examinations revealed an elevated serum IgG4 level of 637 mg/dl in a total IgG level of 2176 mg/dl, and the production of autoantibodies such as rheumatoid factor and antiphospholipid antibodies. In the swollen lymph node, neither clonal proliferation of T or B cells nor Epstein-Barr virus infection was detected, although the structure had been destroyed by the infiltration of lymphocytes and plasma cells. Among total IgG-producing cells, approximately 40% were positive for IgG4. All symptoms and abnormal laboratory findings improved in response to oral prednisolone. Hypocomplementemic urticarial vasculitis may represent a clinical symptom of IgG4-related disease. © 2015 The International Society of Dermatology.

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

    NARCIS (Netherlands)

    Flossmann, Oliver; Berden, Annelies; de Groot, Kirsten; Hagen, Chris; Harper, Lorraine; Heijl, Caroline; Hoglund, Peter; Jayne, David; Luqmani, Raashid; Mahr, Alfred; Mukhtyar, Chetan; Pusey, Charles; Rasmussen, Niels; Stegeman, Coen; Walsh, Michael; Westman, Kerstin

    Background 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. Objective

  1. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    NARCIS (Netherlands)

    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; Harper, Lorraine; Hruskova, 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, Mrten; Stegeman, Coen A.; Tesar, Vladimir; Vaglio, Augusto; Wieczorek, Stefan; Wilde, Benjamin; Zwerina, Jochen; Rees, Andrew J.; Clayton, David G.; Smith, Kenneth G. C.

    2012-01-01

    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

  2. The hairy-print for levamisole-induced vasculitis.

    Science.gov (United States)

    Lazareth, Hélène; Peytavin, Gilles; Polivka, Laura; Dupin, Nicolas

    2012-08-08

    Levamisole-induced vasculitis is a well-characterised antineutrophil cytoplasm antibodies (ANCA)-positive vasculitis in cocaine abuser patients. However, due to the short half-life of levamisole in serum and urine, the causal role of levamisole is not established. Here we report the detection of both levamisole and cocaine in hair samples of a patient who presented with an ANCA-positive vasculitis. The higher concentration of levamisole in proximal sample of the hair confirms that the patient abused of cocaine added with levamisole in the days preceding the development of skin lesions. Although a direct causative role has not been established, our report strongly suggests that levamisole may have triggered vasculitis in this case.

  3. Hypocomplementaemic urticarial vasculitis syndrome: a mimicker of systemic lupus erythematosus.

    Science.gov (United States)

    Roy, Krishnendu; Talukdar, Arunansu; Kumar, Bappaditya; Sarkar, Sumanta

    2013-05-22

    A middle aged female patient presented with generalised palpable purpura associated with intense pruritus along with subconjunctival haemorrhage and orbital inflammation. There was extensive dermographism. Other systemic examinations were within normal limits. Haematological profile was normal except raised D-dimer. Skin biopsy revealed the presence of leucocytoclastic vasculitis. Antinuclear antibody was positive in a titre of 1 : 160, but antidouble-stranded DNA was negative. Urine examination revealed haematuria and proteinuria. Complement C3, C4 and C1q levels were decreased with the presence of anti-C1q antibody. There was a diagnostic dilemma between systemic lupus erythematosus and hypocomplementaemic urticarial vasculitis syndrome. However, as the patient did not fulfil the American College of Rheumatology criteria for systemic lupus erythematosus, but fulfilled all the criteria for hypocomplementaemic urticarial vasculitis syndrome, the case was finally diagnosed as hypocomplementaemic urticarial vasculitis syndrome and treated accordingly with favourable outcome.

  4. A Case of Tuberculous Granulomatous Panniculitis without Vasculitis

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    Yassaman Alipour Tehrany

    2015-07-01

    Full Text Available We report a case of tuberculous granulomatous panniculitis without vasculitis in an 87-year-old female patient with B-cell chronic lymphocytic leukaemia. One month after starting chemotherapy with chlorambucil and prednisone she presented superficial erythematous plaques on the anterior side of the left leg. Three weeks later erythematous painless deep nodules appeared on the left popliteal fossa and on the left thigh. Cutaneous biopsy revealed granulomatous panniculitis without caseation necrosis or vasculitis. Polymerase chain reaction for Mycobacterium tuberculosis revealed positivity in the skin. The final diagnosis was reactivation of latent tuberculosis (TB induced by deep immunosuppression associated with chemotherapy and haematological disease. Tuberculous granulomatous panniculitis without vasculitis is a rare presentation of cutaneous TB and may be part of the heterogeneous histopathologic spectrum of erythema induratum of Bazin (nodular vasculitis. Our case shows that the diagnosis of cutaneous TB requires the correlation of clinical findings with histopathology and microbiological tests.

  5. Lymphomatoid granulomatosis with ocular involvement.

    Science.gov (United States)

    Tse, D T; Mandelbaum, S; Chuck, D A; Nichols, P W; Smith, R E

    1985-01-01

    Lymphomatoid granulomatosis is an angiocentric and angiodestructive lymphoproliferative disorder that involves multiple organs including the lung, skin, kidney and central nervous system. A case of lymphomatoid granulomatosis with ocular involvement is reported herein. Bilateral peripheral retinal vasculitis involving both arterioles and veins, with posterior uveitis, is the prominent clinical finding.

  6. 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....

  7. Clinically Apparent Arterial Thrombosis in Persons with Systemic Vasculitis

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    Alexander Tsoukas

    2017-01-01

    Full Text Available Objective. To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Methods. Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN and granulomatosis with polyangiitis (GPA. Incident acute myocardial infarctions (AMIs and cerebrovascular accidents (CVAs after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data. These were compared to rates of a general population comparator group. The incidences of comorbidities (type 2 diabetes mellitus, dyslipidemia, and hypertension were also collected. Results. Among the 626 patients identified with vasculitis, 19.7% had PAN, 2.9% had Kawasaki disease, 23.8% had GPA, 52.4% had GCA, and 1.3% had Takayasu arteritis. The AMI rate was substantially higher in males aged 18–44 with PAN, with rates up to 268.1 events per 10,000 patient years [95% CI 67.1–1070.2], approximately 30 times that in the age- and sex-matched control group. The CVA rate was also substantially higher, particularly in adults aged 45–65. Patients with vasculitis had elevated incidences of diabetes, dyslipidemia, and hypertension versus the general population. Conclusion. Atherothrombotic rates were elevated in patients identified as having primary systemic vasculitis. While incident rates of cardiovascular comorbidities were also increased, the substantial elevation in AMIs seen in young adults suggests a disease-specific component which requires further investigation.

  8. Clinically Apparent Arterial Thrombosis in Persons with Systemic Vasculitis.

    Science.gov (United States)

    Tsoukas, Alexander; Bernatsky, Sasha; Joseph, Lawrence; Buckeridge, David L; Bélisle, Patrick; Pineau, Christian A

    2017-01-01

    To estimate the incidence rate of clinically apparent arterial thrombotic events and associated comorbidities in patients with primary systemic vasculitis. Using large cohort administrative data from Quebec, Canada, we identified patients with vasculitis, including polyarteritis nodosa (PAN) and granulomatosis with polyangiitis (GPA). Incident acute myocardial infarctions (AMIs) and cerebrovascular accidents (CVAs) after the diagnosis of vasculitis were ascertained in the PAN and GPA group via billing and hospitalization data. These were compared to rates of a general population comparator group. The incidences of comorbidities (type 2 diabetes mellitus, dyslipidemia, and hypertension) were also collected. Among the 626 patients identified with vasculitis, 19.7% had PAN, 2.9% had Kawasaki disease, 23.8% had GPA, 52.4% had GCA, and 1.3% had Takayasu arteritis. The AMI rate was substantially higher in males aged 18-44 with PAN, with rates up to 268.1 events per 10,000 patient years [95% CI 67.1-1070.2], approximately 30 times that in the age- and sex-matched control group. The CVA rate was also substantially higher, particularly in adults aged 45-65. Patients with vasculitis had elevated incidences of diabetes, dyslipidemia, and hypertension versus the general population. Atherothrombotic rates were elevated in patients identified as having primary systemic vasculitis. While incident rates of cardiovascular comorbidities were also increased, the substantial elevation in AMIs seen in young adults suggests a disease-specific component which requires further investigation.

  9. Urticarial vasculitis: a retrospective study of 15 cases.

    Science.gov (United States)

    Moreno-Suárez, F; Pulpillo-Ruiz, Á; Zulueta Dorado, T; Conejo-Mir Sánchez, J

    2013-09-01

    Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis. To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease. We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years. Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus. Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease. Copyright © 2010 Elsevier España, S.L. and AEDV. All rights reserved.

  10. The lung in systemic vasculitis: radiological patterns and differential diagnosis

    Science.gov (United States)

    Mantini, Cesare; Sperandeo, Marco; Galluzzo, Michele; Belcaro, Giovanni; Tartaro, Armando; Cotroneo, Antonio R

    2016-01-01

    The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg–Strauss) and microscopic polyangiitis. Pulmonary involvement is less frequent in immune-complex-mediated small-vessel vasculitides, such as Behçet's disease and Goodpasture's syndrome. Pulmonary involvement associated to large-vessel (gigantocellular arteritis and Takayasu's disease) or medium-vessel (nodose polyarteritis and Kawasaki's disease) vasculitides is extremely rare. The present review describes the main clinical and radiological features of pulmonary vasculitides with major purpose to correlate HRCT findings (solitary or multiple nodules, cavitary lesions, micronodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving”, tracheobronchial involvement, interstitial disease) with pathological results paying particular attention to the description of acute life-threatening manifestations. A thorough medical history, careful clinical examination and the knowledge of radiological patterns are mandatory for a correct and early diagnosis. PMID:26876879

  11. Mycobacterium avium-intracellulare pulmonary infection complicated by cutaneous leukocytoclastic vasculitis in a woman with anorexia nervosa.

    Science.gov (United States)

    Walsh, T L; Baca, V; Stalling, S S; Natalie, A A; Veldkamp, P J

    2014-06-01

    A 53-year-old Caucasian woman with a history of anorexia nervosa developed a bilateral lower extremity rash comprised of palpable red to violaceous, sub-centimeter papular lesions that increased in quantity rapidly. She also noted a 2-month history of non-productive cough. Imaging modalities revealed a thin-walled cavitary lesion in the right lung apex and scattered nodular opacities. Acid fast bacilli (AFB) were found in sputum and subsequently identified by culture as Mycobacterium avium-intracellulare (MAI). Punch biopsies of her skin lesions yielded a histological diagnosis of small-to-medium vessel vasculitis. Stains and cultures for organisms were negative. Her skin lesions resolved quickly after the initiation of antimicrobial therapy for MAI. Hypersensitivity vasculitis associated with an atypical mycobacterial infection is unusual. The postulated underlying mechanism is the deposit of immune complexes and not the bacillus itself. While cutaneous leukocytoclastic vasculitis (CLV) due to MAI is certainly a rare entity, it should be entertained in patients with vasculitic skin lesions and a concomitant pulmonary disease.

  12. Levamisole adulterated cocaine and pulmonary vasculitis: Presentation of two lethal cases and brief literature review.

    Science.gov (United States)

    Karch, Steven B; Busardò, Francesco Paolo; Vaiano, Fabio; Portelli, Francesca; Zaami, Simona; Bertol, Elisabetta

    2016-08-01

    The first case reports of levamisole-related disease in cocaine users were published in 2010, although levamisole adulteration of cocaine was first recognized several years earlier. Currently, more than 70% of street cocaine seizures, in the US and the EU, contain levamisole, which could potentially be converted to aminorex, though the reasons for this practice still remain obscure. Here we report two fatal cases of isolated pulmonary vasculitis in abusers of levamisole-adulterated cocaine, where a complete autopsy, full toxicological analysis by gas chromatography-mass spectrometry (GC-MS) using a previously published method of Karch et al. and histological examination were performed. A control group composed of 11 cases of cocaine related deaths, where the presence of levamisole was excluded in blood, urine and hair, was used. Recent literature on the human pharmacokinetics of levamisole and aminorex is also reviewed. The toxicological analysis revealed positive qualitative and quantitative results for cocaine, benzoylecgonine and levamisole in both cases. In case 1 levamisole was found at the concentration of 13.5 and 61.3mg/L in blood and urine respectively, whereas in case 2 at 17.9 and 70.2mg/L. The histological examination highlighted in case 1 in heart samples microscopic evidence of the typical remodeling changes associated with chronic stimulant abuse, whereas lungs showed numerous lymphocytes surrounding and infiltrating the wall of small pulmonary vessels and a perivascular fibrosis with transforming fibroblasts. In case 2, the myocardial samples showed wide fields of myocardial necrosis characterized by hypercontraction of the myocytes with thickened Z-lines and short sarcomeres, whereas lung samples showed a significant intimal thickening of arteriole walls and lymphocytic infiltration of the wall and edema. Moreover, there were also numerous perivascular lymphocytic infiltrates. Although the pathological cardiac findings have allowed us to establish

  13. Thalidomide in treatment of connective diseases and vasculities

    Directory of Open Access Journals (Sweden)

    D. Ribatti

    2011-09-01

    Full Text Available Thalidomide is an immunomodulatory, anti-inflammatory and anti-angiogenic drug. Thalidomide exerts its effects by decreasing circulating CD4 positive T-cells and stimulating CD8 positive T-cells, by increasing the number of Natural Killer cells and T-helper 2 cells. Thalidomide also inhibits proliferation of stimulated T-cells and leukocyte chemotaxis. It modifies a number of integrin receptors and other leukocytic surface receptors and down-modulates cell-adhesion molecules involved in leukocyte migration. It has been demonstrated that thalidomide inhibits TNFa, IL-5, IL-6, IL-8, IL-12 production and increases production of IL-2, IL-10 and INFg. Moreover thalidomide plays an important role in inhibition of VEGF and FGF-2 mediated angiogenesis. Although the exact mechanism of action is not fully understood and only limited treatment opinions exist, thalidomide plays a role also in connective diseases and vasculities. Thalidomide has been seen efficacious in the treatment of cutaneous disorders in patients with systemic lupus erythematosus and in mucocutaneous disease in Behçet’s disease with a not dose-dependent response, even if it should be restricted to selected patients because of its important side effects.

  14. A novel immunopathological association of IgG4-RD and vasculitis with Hashimoto's thyroiditis.

    Science.gov (United States)

    Minamino, Hiroto; Inaba, Hidefumi; Ariyasu, Hiroyuki; Furuta, Hiroto; Nishi, Masahiro; Yoshimasu, Takashi; Nishikawa, Akinori; Nakanishi, Masanori; Tsuchihashi, Shigeki; Kojima, Fumiyoshi; Murata, Shin-Ichi; Inoue, Gen; Akamizu, Takashi

    2016-01-01

    A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization. The commencement of steroid therapy dramatically exacerbated clinical manifestations including IgG4-RD-associated HT. The measurement of cytokine and chemokine levels as well as FACS analysis in the development of IgG4-RD seemed to be beneficial. In conclusion, an innovative association of HT, IgG4-RD and vasculitis was observed. This report also offers novel diagnostic and therapeutic approaches for IgG4-RD. Recently, a subtype of HT has been considered to be a thyroid manifestation of IgG4-RD, although the etiology of IgG4-RD is not established yet.Immunologically a close association between HT and vasculitis was reported.Leukocytoclastic vasculitis is a rare skin presentation of IgG4-RD.In the current case, during the course of HT, IgG4-RD and leukocytoclastic vasculitis occurred; thus, innate immunity and acquired immunity seem to be involved in the development of IgG4-RD.The measurement of cytokine and chemokines appeared to be beneficial in the development of IgG4-RD.Remarkably, effectiveness of steroid therapy for HT suggested presence of IgG4-RD-associated HT. Therefore, this report highlights the pathogenesis of IgG4-RD and proposes novel therapeutic mechanisms. Clinicians should pay attention to the development of IgG4-RD and vasculitis during long course of HT.

  15. Brief Report: Endothelial-Specific X-Box Binding Protein 1 Deficiency Limits Tumor Necrosis Factor-Induced Leukocyte Recruitment and Vasculitis.

    Science.gov (United States)

    Ziogas, Athanasios; Muders, Michael H; Economopoulou, Matina; Sprott, David; Grossklaus, Sylvia; Siegert, Gabriele; Baretton, Gustavo B; Mitroulis, Ioannis; Chavakis, Triantafyllos

    2015-12-01

    Endothelial cell activation by tumor necrosis factor (TNF) and associated leukocyte infiltration are hallmarks of vasculitis. The aim of this study was to investigate the potential role of the cellular stress-associated endothelial X-box binding protein 1 (XBP-1) transcription factor in TNF-induced endothelial cell inflammation and vasculitis. Mice with an endothelial cell-specific XBP-1 deficiency were used in a modified local Shwartzman reaction (LSR) model of TNF-induced small vessel vasculitis. To address the contribution of XBP-1 to the TNF-mediated inflammatory response in endothelial cells, we examined the activation of XBP-1 expression by TNF as well as the effect of XBP-1 knockdown in endothelial cells on TNF-induced signaling, proinflammatory gene expression, and leukocyte-endothelial cell adhesion. The active spliced form of XBP-1 in endothelial cells was triggered by TNF. In addition, endothelial XBP-1 contributed to the sustained TNF-triggered NF-κB-dependent transcriptional activation of proinflammatory molecules, which was associated with leukocyte-endothelial cell adhesion. In the LSR model, endothelial cell-specific XBP-1-deficient mice displayed significantly less vascular damage, accompanied by reduced perivascular neutrophil infiltration, as compared with wild-type mice. Endothelial XBP-1 is activated by TNF and regulates leukocyte-endothelial cell adhesion in vitro as well as neutrophil infiltration and vascular damage in murine vasculitis. © 2015, American College of Rheumatology.

  16. Review article : The role of CD4(+) T cells in ANCA-associated systemic vasculitis

    NARCIS (Netherlands)

    Abdulahad, Wayel H.; Stegeman, Coen A.; Kallenberg, Cees G. M.

    Antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis (AASV) constitutes a group of primary vasculitides associated with antineutrophil cytoplasmic autoantibodies, which are either directed to proteinase-3 or myeloperoxidase. In contrast to other forms of vasculitis,

  17. Henoch-Schönlein purpura (IgA vasculitis developing after postoperative wound infection by methicillin-resistant Staphylococcus aureus

    Directory of Open Access Journals (Sweden)

    Masataka Satoh

    2016-04-01

    Full Text Available Henoch-Schönlein purpura (HSP is an acute small-vessel leukocytoclastic vasculitis, affecting the skin, joints, gastrointestinal tract and kidneys. Its prognosis depends on the severity of nephritis. A wide variety of pathogens, drugs, and other environmental exposures have been associated with HSP. Although group A β-haemolytic streptococcus has been the most studied, the majority of cases showed no direct link to streptococcal infection. Here we report a case of methicillin-resistant Staphylococcus aureus (MRSA infection-associated HSP. A 68-year-old woman underwent a coronary artery bypass surgery. After the surgery, a postoperative chest wound was infected by MRSA and sternal osteomyelitis developed. Palpable purpura then appeared on the extremities, followed by hematuria, proteinuria and increased serum creatine. Treatments with antibiotics and debridement of the infected wound and sequestrum resulted in rapid improvement of skin symptoms. Renal function partially recovered, however mild hematuria and proteinuria remained. Published work review and the present case suggest that Staphylococcal infection-associated HSP frequently involves kidney disease and its prognosis is likely to be poor compared to a common type of HSP. Further studies are needed to establish an appropriate treatment strategy for Staphylococcal infection-associated HSP.

  18. Hypocomplementemic urticarial vasculitis syndrome: a rare cause of basilar panacinar emphysema.

    Science.gov (United States)

    Pujara, Akshat C; Mohammed, Tan-Lucien H

    2012-05-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare condition characterized by an immune complex-mediated disruption of multiple organ systems. Pulmonary involvement is a major cause of morbidity and mortality in patients with HUVS. HUVS has been described by clinical, histological, and laboratory findings. However, the role of radiography in the diagnosis and management of HUVS has not been established. We examine computed tomography findings in a patient with HUVS and explore the potential of thoracic computed tomography imaging to augment the management of HUVS by serving as a diagnostic tool and marker of disease severity.

  19. Necrotizing Polyarteritis Nodosa-like Vasculitis in a Child with Systemic Lupus Erythematosus.

    Science.gov (United States)

    Nada, Ritambhra; Matthews, Joseph L; Bhattad, Sagar; Gupta, Anju; Singh, Surjit

    2017-02-15

    A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema.

  20. Research vessels

    Digital Repository Service at National Institute of Oceanography (India)

    Rao, P.S.

    by the research vessels RV Gaveshani and ORV Sagar Kanya are reported. The work carried out by the three charted ships is also recorded. A short note on cruise plans for the study of ferromanganese nodules is added...

  1. Musculo-Skeletal and Vascular Involvement in Behcet’s Disease

    Directory of Open Access Journals (Sweden)

    Meryem Can

    2009-12-01

    Full Text Available As a complex vasculitis of unknown etiology, both innate and adaptive immune systems are activated in Behcet’s disease (BD, together with neutrophilic and lympho-histiocytic inflammation. With some features also resembling spondyloarthropathies, arthritis is one of the most frequent manifestations of BD and its prevalence ranges from 40-70%. The most frequently affected joint is knee. Patients generally present with non-erosive, recurrent, asymmetric mono or oligoarthritis. Enthesitis, spondylitis and sacroiilitis are seen less frequently. Non-steroidal anti-inflammatory drugs and intra-articular corticosteroids are the first choice of treatment. Although controlled-trials are lacking, most clinicians also use sulphasalazine and methotrexate in refractory cases. BD, with a prevalence of 25-30%, also involves vessels of all sizes, both arterial and venous. It most commonly affects young males as an important cause of mortality. Endothelial dysfunction is thought to be the major etiopathogenetic factor of vessel involvement. As the disease course continues with remission and relapses and disease activity abating in older ages, controlling inflammation and the prevention of complications at the early phases of the disease is the main aim of treatment. For major vessel involvement cyclophosphamide and high-dose corticosteroids are used, azathiopurine is recommended for small-vessel disease.

  2. Parthenium dermatitis manifesting clinically as polymorphic light eruption and prurigo nodularis- like lesions with vasculitis-like picture on histopathology

    Directory of Open Access Journals (Sweden)

    Chembolli Lakshmi

    2011-01-01

    Full Text Available Parthenium dermatitis is a widespread and distressing dermatoses in rural and urban India caused by the air borne allergen of the Compositae weed Parthenium hysterophorus. Parthenium dermatitis has been thought to be mediated solely by type IV hypersensitivity, but recently a combined immediate (type I and delayed (type IV hypersensitivity mechanism has been postulated in the initiation and perpetuation of parthenium dermatitis, especially in sensitized subjects with an atopic diathesis. Initially, the exposed sites of the body are involved. Later in the course of the disease, unexposed sites may get involved. Various clinical presentations have been described in parthenium dermatitis. Typically, it presents as an air borne contact dermatitis (ABCD involving the eyelids and nasolabial folds Other presentations include a photodermatitis (essentially a pseudo photodermatitis, atopic dermatitis, seborrheic dermatitis, exfoliative dermatitis, hand dermatitis. Photosensitive lichenoid dermatitis and prurigo nodularis are rarer presentations. Uncommon presentations have been described in parthenium dermatitis. They include prurigo nodularis-like lesions and photosensitive lichenoid eruption. Three cases are presented, two of whom presented as polymorphic-like lesions and one as prurigo nodularis. All three patch tested positive to parthenium on Day 2. Prick testing was positive in two of the three patients. Parthenium dermatitis mimicking polymorphic light eruption has not been reported. Histopathology revealed vasculitis in the lesional skin in two of the patients. Although leukocytoclastic vasculitis has been reported earlier from the prick-tested site, this is the first report demonstrating the presence of vasculitis in lesional skin of parthenium dermatitis.

  3. HLA-DR4, DR13(6) and the ancestral haplotype A1B8DR3 are associated with ANCA-associated vasculitis and Wegeners granulomatosis

    NARCIS (Netherlands)

    Stassen, Patricia M.; Cohen-Tervaert, Jan W.; Lems, Simon P. M.; Hepkema, Bouke G.; Kallenberg, Cees G. M.; Stegeman, Coen A.

    Objectives. As the HLA system is involved in recognition of self and non-self, an association with the development of ANCA-associated vasculitis (AAV) seems probable. In this study, the relation between HLA antigens and AAV and its severity were investigated. Methods. Consecutive patients diagnosed

  4. Vancomycin-Induced Leukocytoclastic Vasculitis and Acute Renal Failure Due to Tubulointerstitial Nephritis.

    Science.gov (United States)

    Pingili, Chandra Shekar; Okon, Emmanuel E

    2017-09-25

    BACKGROUND Methicillin-resistant Staphylococcus aureus (MRSA) bacteremia and sepsis are commonly treated with intravenous vancomycin. However, vancomycin treatment is associated adverse reactions, including skin rashes and nephrotoxicity. We present a case of acute renal failure due to acute tubulointerstitial nephritis associated with a diffuse leukocytoclastic vasculitic skin eruption following intravenous vancomycin treatment. CASE REPORT A 79-year-old Caucasian male patient was treated with intravenous vancomycin for MRSA bacteremia. Prior to treatment, his creatinine was normal at 0.6 mg/dl. He presented one week later with shortness of breath, lower limb edema, and acute renal failure. He had a diffuse maculopapular rash involving the trunk and both upper and lower extremities. A renal biopsy and left arm skin biopsy were examined histologically. The skin biopsy showed leukocytoclastic vasculitis. Renal biopsy showed some sclerosed glomeruli, some with mesangial proliferation, and tubulointerstitial inflammation with eosinophils and plasma cells and mild interstitial fibrosis. Although there was some renal arteriolosclerosis, no vasculitic changes were seen, and no vascular thrombosis was present. A diagnosis of leukocytoclastic vasculitis and acute tubulointerstitial nephritis secondary to intravenous vancomycin therapy was made. CONCLUSIONS Although skin reactions associated with drug therapy are common, vancomycin-associated dermal vasculitis is rare. Tubulointerstitial nephritis is also a rare association with vancomycin treatment. This case report has highlighted that patients being treated with intravenous vancomycin should be carefully observed for acute skin rashes and deterioration in renal function, which can be managed by ceasing treatment with vancomycin, steroid challenge, and preventing future exposure to similar antimicrobial agents.

  5. Urinary Soluble CD163 in Active Renal Vasculitis

    NARCIS (Netherlands)

    O'Reilly, Vincent P.; Wong, Limy; Kennedy, Claire; Elliott, Louise A.; O'Meachair, Shane; Coughlan, Alice Marie; O'Brien, Eoin C.; Ryan, Michelle M.; Sandoval, Diego; Connolly, Emma; Dekkema, Gerjan J.; Lau, Jiaying; Abdulahad, Wayel H.; Heeringa, Peter; Buckley, Colm; O'Brien, Cathal; Finn, Stephen; Cohen, Clemens D.; Lindemeyer, Maja T.; Hickey, Fionnuala B.; O'Hara, Paul V.; Feighery, Conleth; Moran, Sarah M.; Mellotte, George; Clarkson, Michael R.; Dorman, Anthony J.; Murray, Patrick T.; Little, Mark A.; Sanders, Johannes

    A specific biomarker that can separate active renal vasculitis from other causes of renal dysfunction is lacking, with a kidney biopsy often being required. Soluble CD163 (sCD163), shed by monocytes and macrophages, has been reported as a potential biomarker in diseases associated with excessive

  6. 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.

  7. 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.

  8. Acute type II cryoglobulinaemic vasculitis mimicking atherosclerotic peripheral vascular disease.

    LENUS (Irish Health Repository)

    Saeed, A

    2012-01-31

    Atherosclerotic peripheral vascular disease is a common presenting cause for digital ischaemia in life long smokers. Acute severe Type II Cryoglobulinaemic vasculitis is a rare yet important cause, which may present with similar clinical features and which if undiagnosed may be rapidly fatal. Following the instigation of therapy with intravenous methylprednisolone and cyclophosphamide this patient made an excellent recovery.

  9. Surface microscopy for discriminating between common urticaria and urticarial vasculitis.

    Science.gov (United States)

    Vázquez-López, F; Maldonado-Seral, C; Soler-Sánchez, T; Perez-Oliva, N; Marghoob, A A

    2003-09-01

    Urticarial vasculitis is a clinicopathological entity that overlaps with common urticaria, and biopsy is required for differentiation between them. To determine, for the first time, if skin surface microscopy can aid in the clinical differentiation between common urticaria and urticarial vasculitis in daily practice. Lesions in 20 consecutive patients with a clinical diagnosis of urticaria were studied by biopsy and skin surface microscopy (10x dermoscope) after covering the lesions with olive oil. Lesions were photographed with Dermaphot equipment. A biopsy was taken from all patients. Statistical analysis included Fisher's exact test and Cohen kappa statistics (intra-observer reproducibility). Two dermoscopic patterns were observed: (i). a red-lined vascular pattern (17/20 patients); and (ii). a purpuric globular pattern (3/20 patients). Leucocytoclastic vasculitis was demonstrated histologically in all lesions presenting purpuric globules (3/3) but in none of the lesions presenting a dermoscopic red-lined pattern (P urticarial lesions, and that purpuric globules indicate underlying leucocytoclastic vasculitis.

  10. Effectiveness of omalizumab in a case of urticarial vasculitis.

    Science.gov (United States)

    Fueyo-Casado, A; Campos-Muñoz, L; González-Guerra, E; Pedraz-Muñoz, J; Cortés-Toro, J A; López-Bran, E

    2017-03-01

    Urticarial vasculitis (UV) is a mainly leucocytoclastic vasculitis with urticarial plaques. Treating these patients is challenging as the available treatments have poor efficacy. Oral corticosteroids are considered the first-line treatment, but H1 antihistamines, dapsone, colchicine, antimalarials, ciclosporin and antileucotrienes have all been tried also. However, because of their adverse effects and/or lack of efficacy, new agents are still needed. Omalizumab, an anti-IgE antibody, shows efficacy in chronic spontaneous urticaria, and might also be a good treatment for angio-oedema and urticarial vasculitis. To our knowledge, there have been only seven relevant case reports published in the English literature. We add a new case of severe chronic recurrent urticarial vasculitis refractory to all of the drugs mentioned above. We started the patient on subcutaneous omalizumab 300 mg every 4 weeks, which produced clinical improvement within the first month and total remission in the fifth month. The patient has remained stable for 23 months, and follow-up is ongoing. © 2017 British Association of Dermatologists.

  11. Long- term outcome of paediatric patients with ANCA vasculitis

    Directory of Open Access Journals (Sweden)

    Pusey Charles D

    2011-06-01

    Full Text Available Abstract Background Primary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis. Methods The adult patients in our vasculitis clinics who had presented in childhood, with a follow up time of greater than 10 years were included. We also reviewed the literature for articles describing the clinical outcome of paediatric patients with ANCA vasculitis. Results We describe the clinical course of 8 adults who presented in childhood with ANCA vasculitis. 7 patients had Wegener's granulomatosis and 1 had microscopic polyangiitis. The median age at presentation was 11.5 years, and follow up time ranged form 11 to 30 years. Induction therapy for all patients was steroids and/or cyclophosphamide. Maintenance therapy was with azathioprine or mycophenolate mofetil. Biological agents were used in 3 patients for relapsed disease in adulthood only. Seven patients achieved complete remission. All patients experienced disease relapse, with a median of 4 episodes. Kidney function was generally well preserved, with median eGFR 76 ml/min. Only one patient developed end-stage renal failure and one patient died after 25 years of disease. Treatment-related morbidity rates were high; 7 suffered from infections, 4 were infertile, 2 had skeletal complications, and 1 developed malignancy. Conclusion Close long- term follow up of paediatric patients with ANCA vasculitis is imperative, as this patient cohort is likely to live long enough to develop significant treatment and disease- related morbidities. Prospective cohort studies with novel therapies including paediatric patients are crucial to help us determine the best approach to managing this complex group

  12. Vasculitis: Molecular Imaging by Targeting the Inflammatory Enzyme Myeloperoxidase

    Science.gov (United States)

    Su, Henry S.; Nahrendorf, Matthias; Panizzi, Peter; Breckwoldt, Michael O.; Rodriguez, Elisenda; Iwamoto, Yoshiko; Aikawa, Elena; Weissleder, Ralph

    2012-01-01

    Purpose: To determine if a molecular imaging approach targeting the highly oxidative enzyme myeloperoxidase (MPO) can help noninvasively identify and confirm sites of vascular wall inflammation in a murine model of vasculitis. Materials and Methods: Animal experiments were approved by the institutional animal care committee. Twenty-six mice were studied, including eight MPO-deficient and six sham-operated mice as controls. Vasculitis was induced with intraperitoneal injection of Candida albicans water-soluble fraction (CAWS). Aortic root magnetic resonance imaging was performed after intravenous injection of the activatable MPO sensor (bis-5-hydroxytryptamide-diethylenetriaminepentatacetate gadolinium) (n = 23), referred to as MPO-Gd, or gadopentetate dimeglumine (n = 10). Seven mice were randomly assigned to receive either MPO-Gd or gadopentetate dimeglumine first. Aortic root specimens were collected for biochemical and histopathologic analyses to validate imaging findings. Statistical significance was calculated for contrast-to-noise ratios (CNRs) by using the paired t test. Results: In the aortic root, the mean MPO-Gd CNRs after agent injection (CNR = 28.1) were more than 2.5-fold higher than those of sham-operated mice imaged with MPO-Gd and vasculitis mice imaged with gadopentetate dimeglumine (CNR = 10.6) (P gadopentetate dimeglumine. Histopathologic and biochemical analyses for MPO and myeloid cells confirmed imaging findings. In MPO-deficient mice, injection of CAWS did not result in a vasculitis phenotype, implying a key role of the imaging target in disease cause. Conclusion: Molecular imaging targeting MPO can be a useful biomarker to noninvasively detect and confirm inflammation in vasculitis by using a murine model of Kawasaki disease. © RSNA, 2011 Supplemental material: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.11110040/-/DC1 PMID:22084204

  13. Multiple aneurysms and gastrointestinal involvement in Behcet's disease: A case report.

    Science.gov (United States)

    Wang, Mengdi; Sun, Weiwei; Chen, Zhenjie; Wang, Xiaona; Lv, Jie; Tan, Quanming; Wang, Yaoxian; Zhou, Jingwei

    2017-12-01

    Behcet's disease (BD) is a rare systemic vasculitis disorder that can involve vessels of any size. A 60-year-old female had recurrent painful mouth ulcers about 30 years ago and recently presented with abdominal distension, conjunctival congestion, and chest pain in sequence. The patient was diagnosed with BD according to the International Criteria for Behcet's Disease (ICBD). A therapy of glucocorticoids and cyclophosphamide was administered. After a 3-month treatment, follow-up examination showed a depression of the inflammation and a slight decrease of the arterial aneurysms. BD patients may get a delayed diagnosis because of the long duration between the first and subsequent manifestations. Suspect patients should be followed-up and the diagnosis of BD should be considered when multiple tissues or organs are involved.

  14. Simultaneous automated screening and confirmatory testing for vasculitis-specific ANCA.

    Directory of Open Access Journals (Sweden)

    Mandy Sowa

    Full Text Available Anti-neutrophil cytoplasmic antibodies (ANCA are the serological hallmark of small vessel vasculitis, so called ANCA-associated vasculitis. The international consensus requires testing by indirect immunofluorescence (IIF on human ethanol-fixed neutrophils (ethN as screening followed by confirmation with enzyme-linked immunosorbent assays (ELISAs. This study evaluates the combination of cell- and microbead-based digital IIF analysis of ANCA in one reaction environment by the novel multiplexing CytoBead technology for simultaneous screening and confirmatory ANCA testing. Sera of 592 individuals including 118 patients with ANCA-associated vasculitis, 133 with rheumatoid arthritis, 49 with infectious diseases, 77 with inflammatory bowel syndrome, 20 with autoimmune liver diseases, 70 with primary sclerosing cholangitis and 125 blood donors were tested for cytoplasmic ANCA (C-ANCA and perinuclear ANCA (P-ANCA by classical IIF and ANCA to proteinase 3 (PR3 and myeloperoxidase (MPO by ELISA. These findings were compared to respective ANCA results determined by automated multiplex CytoBead technology using ethN and antigen-coated microbeads for microbead immunoassays. There was a good agreement for PR3- and MPO-ANCA and a very good one for P-ANCA and C-ANCA by classical and multiplex analysis (Cohen's kappa [κ] = 0.775, 0.720, 0.876, 0.820, respectively. The differences between classical testing and CytoBead analysis were not significant for PR3-ANCA, P-ANCA, and C-ANCA (p<0.05, respectively. The prevalence of confirmed positive ANCA findings by classical testing (IIF and ELISA compared with multiplex CytoBead analysis (IIF and microbead immunoassay positive resulted in a very good agreement (κ = 0.831 with no significant difference of both methods (p = 0.735. Automated endpoint-ANCA titer detection in one dilution demonstrated a very good agreement with classical analysis requiring dilution of samples (κ = 0.985. Multiplexing by Cyto

  15. Accompanying conditions in patients with chronic spontaneous urticaria and urticarial vasculitis: Results of a retrospective study

    Directory of Open Access Journals (Sweden)

    Sevgi Akarsu

    2015-03-01

    Full Text Available Background and Design: Chronic spontaneous urticaria (CSU, the most common form of chronic urticaria, is characterized by spontaneous wheals and/or angioedema lasting longer than six weeks. Urticarial vasculitis (UV is a small vessel vasculitis; but is also included in the various classification systems of chronic urticaria by some authors. The aim of our study was to evaluate the frequency of accompanying conditions, and to compare the demographic, clinical and laboratory features of patients with CSU and UV. Materials and Methods: In this study, the files of CSU (n=146 and UV (n=43 patients who had been hospitalized between January 2010-December 2013 were retrospectively reviewed in terms of demographic data, disease-specific history, accompanying diseases, medications and laboratory findings. Results: When comparing between the two groups for statistical differences, it was found that the frequencies of personal atopy and angioedema were significantly higher in patients with CSU; however, the patients with UV had significantly higher rates for female gender and the presence of systemic symptoms compared to those with CSU. It was determined that the mean values for erythrocyte sedimentation rate and C-reactive protein, as well as the rates of antinuclear antibody positivity and hypocomplementemia were significantly higher in the UV group, compared with those in the CSU group. Accompanying factors were determined as infections, medications, autoimmune diseases and malignities in order of frequency in both CSU and UV groups, but only the frequency of autoimmune diseases was significantly higher in patients with UV group. Conclusion: Although various authors have reported that a large number (up to 40-60% of patients with UV may present only with wheals (clinically indistinguishable from CSU; we assumed that it would be more appropriate to be considered UV as a priority in patients especially who had systemic symptom, autoimmune disease

  16. Enhancing supply vessel safety

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2002-07-01

    A supply-vessel bridge installation consists of a navigating bridge and a control position aft, from which operators control the ship when close to rigs or platforms, and operate winches and other loading equipment. The international Convention for Safety of I Ale at Sea (SOLAS) does not regulate the layout, so design varies to a large degree, often causing an imperfect working environment. As for other types of ships, more than half the offshore service vessel accidents at sea are caused by bridge system failures. A majority can be traced back to technical design, and operational errors. The research and development project NAUT-OSV is a response to the offshore industry's safety concerns. Analysis of 24 incidents involving contact or collision between supply vessels and offshore installations owned or operated by Norwegian companies indicated that failures in the bridge system were often the cause.

  17. The challenge of treating hepatitis C virus-associated cryoglobulinemic vasculitis in the era of anti-CD20 monoclonal antibodies and direct antiviral agents.

    Science.gov (United States)

    Roccatello, Dario; Sciascia, Savino; Rossi, Daniela; Solfietti, Laura; Fenoglio, Roberta; Menegatti, Elisa; Baldovino, Simone

    2017-06-20

    Mixed cryoglobulinemia syndrome (MC) is a systemic vasculitis involving kidneys, joints, skin, and peripheral nerves. While many autoimmune, lymphoproliferative, and neoplastic disorders have been associated with this disorder, hepatitis C virus (HCV) is known to be the etiologic agent in the majority of patients. Therefore, clinical research has focused on anti-viral drugs and, more recently, on the new, highly potent Direct-acting Antiviral Agents (DAAs). These drugs assure sustained virologic response (SVR) rates >90%. Nevertheless, data on their efficacy in patients with HCV-associated cryoglobulinemic vasculitis are disappointing, possibly due to the inability of the drugs to suppress the immune-mediated process once it has been triggered.Despite the potential risk of exacerbation of the infection, immunosuppression has traditionally been regarded as the first-line intervention in cryoglobulinemic vasculitis, especially if renal involvement is severe. Biologic agents have raised hopes for more manageable therapeutic approaches, and Rituximab (RTX), an anti CD20 monoclonal antibody, is the most widely used biologic drug. It has proved to be safer than conventional immunosuppressants, thus substantially changing the natural history of HCV-associated cryoglobulinemic vasculitis by providing long-term remission, especially with intensive regimens.The present review focuses on the new therapeutic opportunities offered by the combination of biological drugs, mainly Rituximab, with DAAs.

  18. Patients with ANCA-associated vasculitis admitted to the intensive care unit with acute vasculitis manifestations: a retrospective and comparative multicentric study.

    Science.gov (United States)

    Demiselle, Julien; Auchabie, Johann; Beloncle, François; Gatault, Philippe; Grangé, Steven; Du Cheyron, Damien; Dellamonica, Jean; Boyer, Sonia; Beauport, Dimitri Titeca; Piquilloud, Lise; Letheulle, Julien; Guitton, Christophe; Chudeau, Nicolas; Geri, Guillaume; Fourrier, François; Robert, René; Guérot, Emmanuel; Boisramé-Helms, Julie; Galichon, Pierre; Dequin, Pierre-François; Lautrette, Alexandre; Bollaert, Pierre-Edouard; Meziani, Ferhat; Guillevin, Loïc; Lerolle, Nicolas; Augusto, Jean-François

    2017-12-01

    Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce. We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group. In the ICU group, patients predominantly showed granulomatosis with polyangiitis and proteinase-3 ANCAs. Compared with the non-ICU group, the ICU group showed comparable Birmingham vasculitis activity score and a higher frequency of heart, central nervous system and lungs involvements. Respiratory assistance, renal replacement therapy and vasopressors were required in 68.0, 56.7 and 26.8% of ICU patients, respectively. All but one patient (99%) received glucocorticoids, 85.6% received cyclophosphamide, and 49.5% had plasma exchanges as remission induction regimens. Fifteen (15.5%) patients died during the ICU stay. The following were significantly associated with ICU mortality in the univariate analysis: the need for respiratory assistance, the use of vasopressors, the occurrence of at least one infection event in ICU, cyclophosphamide treatment, sequential organ failure assessment at admission and simplified acute physiology score II. After adjustment on sequential organ failure assessment or infection, cyclophosphamide was no longer a risk factor for mortality. Despite a higher initial mortality rate of ICU patients within the first hospital stay (p < 0.0001), the long-term mortality of hospital survivors did not differ between ICU and non-ICU groups (18.6 and 20.4%, respectively, p = 0.36). Moreover, we observed no renal survival difference between groups after a 1-year follow-up (82.1 and 80.5%, p = 0.94). This study supports the idea that experiencing an ICU challenge does not impact the long-term prognosis of

  19. [Severe occlusive bilateral retinal vasculitis within the scope of seronegative systemic lupus erythematosus].

    Science.gov (United States)

    Koch, J W; al Nawaiseh, I; Koch, F H

    1992-11-01

    Retinal vascular manifestation is the most common form of ophthalmic involvement in patients with systemic lupus erythematosus (SLE). Most frequently these consist of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vaso-occlusive disease, termed retinal vasculitis, has been described. We report on a 37-year-old white female with a 13-year follow-up of chronic discoid lupus erythematosus, which suffered massive bilateral visual loss coincident with the systemic exacerbation of her disease (proteinuria, pneumonia, serositis, leucopenia). The diagnosis of SLE was established with reference to the revised ARA-criteria (American Rheumatism Association). Ophthalmoscopy and fluorescein angiography revealed the typical aspect of a SLE-associated vaso-occlusive retinopathy on both eyes with marked ischemia of the macula. Immediate maximal immuno-suppressive therapy, early performed panretinal photocoagulation and subsequent cryoretinopexy did not stop the progression of the disease. Six months after the initial event vascularisations of the disc and rubeosis iridis occurred, but no secondary glaucoma up to date. In this patient, the almost complete absence of characteristic autoantibodies and immunological markers was striking. The correlation with other lupus manifestations, different therapeutic concepts and prognostic factors in SLE-associated retinal vasculitis are discussed.

  20. Individualization of the ANCA associated vasculitis treatment

    Directory of Open Access Journals (Sweden)

    Zbigniew Zdrojewski

    2014-09-01

    Full Text Available Vasculitides associated with the presence of anti-neutrophil cytoplasmic antibodies (AAV are the group of systemic diseases characterized by necrotizing inflammation of small and medium size vessels, without immunologic deposits and presence of circulating specific antibodies. Recent genetics and cohorts study should improve comprehension of pathogenesis of AAV, stratification patients in homogenous group and should progress therapeutic implication. Immunosuppressive therapy of AAV includes two major periods: first induction of remission, second maintenance therapy. It should be dedicated individually not only depending on the stage and severity of the disease but also on genetic and some prognostic factors. Previous randomized trials and clinical observations show some possible limitations of treatment with cyclophosphamide and steroids. Rituximab seems to be a good alternative in those patients in induction therapy as well as in maintenance therapy.

  1. Cocaine-induced vasculitis: is this a new trend?

    Directory of Open Access Journals (Sweden)

    García Pérez MR

    2013-10-01

    Full Text Available Miraida Reneé García Pérez,1 Vanessa L Ortiz-González,1 Maria Betancourt,1 Rogelio Mercado21Department of Internal Medicine, San Juan City Hospital, 2Department of Dermatology, University of Puerto Rico School of Medicine, San Juan, Puerto RicoAbstract: Cocaine-induced vasculitis is a rare complication found in drug abusers. It occurs due to cocaine adulterated with levamisole. Levamisole was once used as a chemotherapy and immunomodulator for different conditions. One of the side effects of this medication is necrotizing vasculitis which has been reported in the US and Puerto Rico. Here we present another case of cocaine induced vasculitis in Puerto Rico. We describe a 43-year-old female with past medical history of bronchial asthma, migraine, and crack smoking who presented to the emergency room due to blood in her urine for 5 days. She also reported fever, chills, and fatigue. At the physical exam she had a right knee ulcer with swelling erythema, warmth, and pain. Also, she had retiform purpuric plaque lesions in her ears, bilaterally. Eroded plaques with elevated borders at left foot and finger dorsum were also present. Laboratory workup was positive for cocaine. The patient showed leucopenia and microcytic anemia with a normal absolute neutrophil count in her cell blood count. Blood cultures, urine cultures, and ulcer cultures were negative. Urinalysis was positive for proteinuria and hematuria. Also, the patient had positive perinuclear anti-neutrophil cytoplasmic antibody, cytoplasmic anti-neutrophil cytoplasmic antibody, and antinuclear antibody tests and elastase specificity. She showed negative anticardiolipin and lupus anticoagulant antibodies. Her complement levels were decreased. The punch biopsy of her ear showed superficial thrombosis of superficial vascular plexus with perivascular lymphocytic infiltrates and deeper sections showed epidermal necrosis and necrotizing vasculitis. She was started on a high dose of steroids, but

  2. Present and future management of anti-neutrophil cytoplasmic antibody associated vasculitis: how therapy changed the prognosis

    Directory of Open Access Journals (Sweden)

    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.

  3. Portal vein resection and reconstruction with artificial blood vessels is safe and feasible for pancreatic ductal adenocarcinoma patients with portal vein involvement: Chinese center experience.

    Science.gov (United States)

    Xie, Zhi-Bo; Gu, Ji-Chun; Zhang, Yi-Fan; Yao, Lie; Jin, Chen; Jiang, Yong-Jian; Li, Ji; Yang, Feng; Zou, Cai-Feng; Fu, De-Liang

    2017-09-29

    Evidence shows that portal vein resection (PVR) increase the resectability but does little benefit to overall survival in all pancreatic ductal adenocarcinoma (PDAC) patients. But for patients with portal vein involvement, PVR is the only radical choice. But whether the PDAC patients with portal vein involvement would benefit from radical pancreaticoduodenectomy with PVR or not is controversial. All 204 PDAC patients with portal vein involvement were enrolled in this study [PVR group, n=106; surgical bypass (SB) group, n=52; chemotherapy group, n=46]. Overall survival and prognostic factors were analyzed among three groups. Moreover, a literature review of 13 studies were also conducted. Among 3 groups, patients in PVR group achieved a significant longer survival (median survival: PVR group, 22.83 months; SB group, 7.26 months; chemotherapy group, 10.64 months). Therapy choice [hazard ratio (HR) =1.593, 95% confidence interval (CI) 1.323 to 1.918, Pportal vein involvement.

  4. Brucella peritonitis and leucocytoclastic vasculitis due to Brucella melitensis

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    Murat Dizbay

    Full Text Available Brucellosis is a multisystemic disease that rarely leads to a fatal outcome. While reticuloendothelial system organs are mostly affected, peritonitis and posthepatitic cirrhosis are also complications of brucellosis, though they are very rare. Brucella spp. can also trigger immunological reactions. We report a case of brucellosis with peritonitis, renal failure and leucocytoclastic vasculitis caused by Brucella melitensis, which led to a fatal outcome. Brucellosis should be considered in the differential diagnosis of vasculitic diseases, especially in endemic areas.

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

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    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.

  6. Pulmonary hemosiderosis with normocomplementemic urticarial vasculitis in a child.

    Science.gov (United States)

    Yuksel, H; Yilmaz, O; Savas, R; Kirmaz, C; Sogut, A; Ozalp, S

    2007-03-01

    Pulmonary hemosiderosis is rarely associated with urticarial vaculitis especially if normocomplementemic. An eigth year old girl presented with relapsing and remitting chronic and persistent urticarial lesions, conjunctival injection, recurrent cough and hemoptysis. Respiratory findings started at seven years of age. Physical examination revealed diffuse skin lesions mainly settled on the extremities, non-purulent conjunctival injection, rare ronchi and fine crackles in bilateral lower zones of the lungs. Biopsy of the urticaria like skin lesions demonstrated leukocytoclastic vasculitis. Rheumatological markers were negative. Levels of complement fractions 3 and 4 were normal. Chest x-ray demonstrated diffuse alveolar infiltrative images. High Resolution Computed Tomography of the chest revealed diffuse ground-glass appearance, increased interstitial density. Diagnostic flexible fiberoptic bronchoscopy was performed and bronchoalveolar lavage fluid revealed hemosiderin laden alveolar macrophages. She was started on systemic corticosteroid treatment. During follow up, pulmonary symptoms disappeared, however skin lesions and conjunctival symptoms persisted and exacerbated four times in two years. CT of lungs after two years of treatment revealed rare patchy areas of ground glass appearance in bilateral lower lobes and right upper lobe as well as a few of millimetric pleural nodules. This patient is still followed up under low dose steroids and pulmonary findings regressed but low grade inflammation due to vasculitis is thought to continue as supported by the persistence of tomographic findings in the lungs despite the absence of any symptoms. This case demonstrates association of urticarial vasculitis and pulmonary hemosiderosis in the setting of normocomplementemia.

  7. Neonatal cerebral oxygenation is not linked to foetal vasculitis and predicts intraventricular haemorrhage in preterm infants

    DEFF Research Database (Denmark)

    Sorensen, Line C; Maroun, Lisa L; Borch, Klaus

    2008-01-01

    Aim: The aim of the study was to compare the cerebral tissue oxygenation index (c-TOI) measured by near infrared spectroscopy (NIRS) in infants with and without foetal vasculitis. Methods: Twenty-four infants with placental signs of a foetal inflammatory response (FIR), foetal vasculitis, were co.......002). Conclusion: Cerebral oxygenation was not affected in the first day of life in preterm infants born with foetal vasculitis, while cerebral oxygenation in infants that later developed intraventricular haemorrhage was impaired....

  8. Levamisole-induced leukocytoclastic vasculitis and neutropenia in a patient with cocaine use: An extensive case with necrosis of skin, soft tissue, and cartilage

    Science.gov (United States)

    2012-01-01

    Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month’s duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography–mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390

  9. Current and emerging treatment options for ANCA-associated vasculitis: potential role of belimumab and other BAFF/APRIL targeting agents

    Directory of Open Access Journals (Sweden)

    Lenert A

    2015-01-01

    Full Text Available Aleksander Lenert,1 Petar Lenert21Division of Rheumatology, University of Kentucky, Kentucky Clinic, Lexington, KY, USA; 2Division of Immunology, Department of Internal Medicine, The University of Iowa, Iowa City, IA, USAAbstract: Antineutrophil cytoplasmic antibody (ANCA-associated vasculitis (AAV comprises several clinical entities with diverse clinical presentations, outcomes, and nonunifying pathogenesis. AAV has a clear potential for relapses, and shows unpredictable response to treatment. Cyclophosphamide-based therapies have remained the hallmark of induction therapy protocols for more than four decades. Recently, B-cell depleting therapy with the anti-CD20 antibody rituximab has proved beneficial in AAV, leading to Food and Drug Administration approval of rituximab in combination with corticosteroids for the treatment of AAV in adults. Rituximab for ANCA-associated vasculitis and other clinical trials provided clear evidence that rituximab was not inferior to cyclophosphamide for remission induction, and rituximab appeared even more beneficial in patients with relapsing disease. This raised hopes that other B-cell-targeted therapies directed either against CD19, CD20, CD22, or B-cell survival factors, B-cell activating factor of the tumor necrosis factor family (BAFF and a proliferation-inducing ligand could also be beneficial for the management of AAV. BAFF neutralization with the fully humanized monoclonal antibody belimumab has already shown success in human systemic lupus erythematosus and, along with another anti-BAFF reagent blisibimod, is currently undergoing Phase II and III clinical trials in AAV. Local production of BAFF in granulomatous lesions and elevated levels of serum BAFF in AAV provide a rationale for BAFF-targeted therapies not only in AAV but also in other forms of vasculitis such as Behcet’s disease, large-vessel vasculitis, or cryoglobulinemic vasculitis secondary to chronic hepatitis C infection. BAFF

  10. Severe leukocytoclastic vasculitis secondary to the use of a naproxen and requiring amputation: a case report

    Directory of Open Access Journals (Sweden)

    Martin Jeanine

    2010-07-01

    Full Text Available Abstract Introduction Leukocytoclastic vasculitis (also known as hypersensitivity vasculitis and cutaneous necrotizing vasculitis can present with various manifestations, which often delays the diagnosis and treatment. In order to show the importance of the early recognition of leukocytoclastic vasculitis, we present a case which occurred secondary to the use of a common pharmaceutical, naproxen. We were unable to find a case of leukocytoclastic vasculitis secondary to naproxen in the literature. Case presentation We present the case of a 33-year-old African American woman with below the knee and bilateral digital gangrene from hypersensitivity vasculitis secondary to the non-steroidal anti-inflammatory medication naproxen. Conclusion This is an original case report focusing on the rheumatologic management of leukocytoclastic vasculitis. However, other specialties, such as internal medicine, dermatology, infectious disease, general surgery and pathology, can gain valuable information by reviewing this case report. Reporting a case of leukocytoclastic vasculitis secondary to treatment with naproxen will advance our understanding of this disease etiology by adding yet another non-steroidal anti-inflammatory drug to the list of potential causes of leukocytoclastic vasculitis.

  11. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients.

    Science.gov (United States)

    Jachiet, Marie; Flageul, Béatrice; Deroux, Alban; Le Quellec, Alain; Maurier, François; Cordoliani, Florence; Godmer, Pascal; Abasq, Claire; Astudillo, Leonardo; Belenotti, Pauline; Bessis, Didier; Bigot, Adrien; Doutre, Marie-Sylvie; Ebbo, Mikaël; Guichard, Isabelle; Hachulla, Eric; Héron, Emmanuel; Jeudy, Géraldine; Jourde-Chiche, Noémie; Jullien, Denis; Lavigne, Christian; Machet, Laurent; Macher, Marie-Alice; Martel, Clotilde; Melboucy-Belkhir, Sara; Morice, Cécile; Petit, Antoine; Simorre, Bernard; Zenone, Thierry; Bouillet, Laurence; Bagot, Martine; Frémeaux-Bacchi, Véronique; Guillevin, Loïc; Mouthon, Luc; Dupin, Nicolas; Aractingi, Selim; Terrier, Benjamin

    2015-02-01

    Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology that is rarely described in the literature. We undertook this study to analyze the clinical spectrum and the therapeutic management of patients with HUV. We conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histologic leukocytoclastic vasculitis, and hypocomplementemia. We assessed clinical and laboratory data and evaluated the patients' cutaneous and immunologic responses to therapy. We evaluated treatment efficacy by measuring the time to treatment failure. Urticarial lesions were typically more pruritic than painful and were associated with angioedema in 51% of patients, purpura in 35%, and livedo reticularis in 14%. Extracutaneous manifestations included constitutional symptoms (in 56% of patients) as well as musculoskeletal involvement (in 82%), ocular involvement (in 56%), pulmonary involvement (in 19%), gastrointestinal involvement (in 18%), and kidney involvement (in 14%). Patients with HUV typically presented with low C1q levels and normal C1 inhibitor levels, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine or colchicine seemed to be as effective as corticosteroids as first-line therapy. In patients with relapsing and/or refractory disease, rates of cutaneous and immunologic response to therapy seemed to be higher with conventional immunosuppressive agents, in particular, azathioprine, mycophenolate mofetil, or cyclophosphamide, while a rituximab-based regimen tended to have higher efficacy. Finally, a cutaneous response to therapy was strongly associated with an immunologic response to therapy. HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly, musculoskeletal and ocular involvement associated with anti-C1q antibodies, which were found in approximately half of the patients. The best strategy for treating HUV has yet to be defined

  12. Circulating ADAM17 Level Reflects Disease Activity in Proteinase-3 ANCA-Associated Vasculitis

    Science.gov (United States)

    Bertram, Anna; Lovric, Svjetlana; Engel, Alissa; Beese, Michaela; Wyss, Kristin; Hertel, Barbara; Park, Joon-Keun; Becker, Jan U.; Kegel, Johanna; Haller, Hermann; Haubitz, Marion

    2015-01-01

    ANCA-associated vasculitides are characterized by inflammatory destruction of small vessels accompanied by enhanced cleavage of membrane-bound proteins. One of the main proteases responsible for ectodomain shedding is disintegrin and metalloproteinase domain-containing protein 17 (ADAM17). Given its potential role in aggravating vascular dysfunction, we examined the role of ADAM17 in active proteinase-3 (PR3)-positive ANCA-associated vasculitis (AAV). ADAM17 concentration was significantly increased in plasma samples from patients with active PR3-AAV compared with samples from patients in remission or from other controls with renal nonvascular diseases. Comparably, plasma levels of the ADAM17 substrate syndecan-1 were significantly enhanced in active AAV. We also observed that plasma-derived ADAM17 retained its specific proteolytic activity and was partly located on extracellular microparticles. Transcript levels of ADAM17 were increased in blood samples of patients with active AAV, but those of ADAM10 or tissue inhibitor of metalloproteinases 3, which inhibits ADAMs, were not. We also performed a microRNA (miR) screen and identified miR-634 as significantly upregulated in blood samples from patients with active AAV. In vitro, miR-634 mimics induced a proinflammatory phenotype in monocyte-derived macrophages, with enhanced expression and release of ADAM17 and IL-6. These data suggest that ADAM17 has a prominent role in AAV and might account for the vascular complications associated with this disease. PMID:25788529

  13. Efficacy and Safety of Sofosbuvir Plus Daclatasvir for Treatment of HCV-Associated Cryoglobulinemia Vasculitis.

    Science.gov (United States)

    Saadoun, David; Pol, Stanislas; Ferfar, Yasmina; Alric, Laurent; Hezode, Christophe; Si Ahmed, Si Nafa; de Saint Martin, Luc; Comarmond, Cloé; Bouyer, Anne Sophie; Musset, Lucile; Poynard, Thierry; Resche Rigon, Matthieu; Cacoub, Patrice

    2017-07-01

    Circulating mixed cryoglobulins are detected in 40%-60% of patients with hepatitis C virus (HCV) infection, and overt cryoglobulinemia vasculitis (CryoVas) develops in approximately 15% of patients. Remission of vasculitis has been associated with viral clearance, but few studies have reported the effectiveness of direct-acting antiviral drugs in these patients. We performed an open-label, prospective, multicenter study of the effectiveness and tolerance of an all-oral, interferon- and ribavirin-free regimen of sofosbuvir plus daclatasvir in patients with HCV-associated CryoVas. Forty-one consecutive patients with active HCV-associated CryoVas (median age, 56 y; 53.6% women) were recruited from hospitals in Paris, France, from 2014 through 2016. They received sofosbuvir (400 mg/day) plus daclatasvir (60 mg/day) for 12 weeks (n = 32) or 24 weeks (n = 9), and were evaluated every 4 weeks until week 24 and at week 36. Blood samples were analyzed for complete blood count, serum chemistry profile, level of alanine aminotransferase, rheumatoid factor activity, C4 fraction of complement, and cryoglobulin; peripheral blood mononuclear cells were isolated for flow cytometry analysis. Thirty-seven patients (90.2%) had a complete clinical response (defined by improvement of all the affected organs involved at baseline and no clinical relapse) after a median time of 12 weeks of therapy; all had a sustained virologic response (no detectable serum HCV RNA 12 weeks after the end of antiviral therapy). Patients' mean cryoglobulin level decreased from 0.56 ± 0.18 at baseline to 0.21 ± 0.14 g/L at week 36, and no cryoglobulin was detected in 50% of patients at this time point. After antiviral therapy, patients had increased numbers of T-regulatory cells, IgM+CD21-/low-memory B cells, CD4+CXCR5+ interleukin 21+ cells, and T-helper 17 cells, compared with before therapy. After a median follow-up period of 26 months (interquartile range, 20-30 mo), no patients had a serious adverse

  14. Facial ulcerations due to Acinetobacter baumannii: Vessel thrombosis with bacterial mycelia

    Directory of Open Access Journals (Sweden)

    Dong Ming Li

    2014-01-01

    Full Text Available A 14-year-old girl presented with a 2-week history of progressive facial ulcerations that did not respond to cephalexin and topical dexamethasone. Biopsy on the ulcer showed rod-shaped bacteria and actinomycetes-like mycelia in the vessel walls and within thrombi. Tissue culture yielded Acinetobacter baumannii, which was resistant to cephalexin. A favourite outcome was achieved with minocycline treatment. This is the first case report of A. baumannii-related vasculitis.

  15. Necrotizing leptomeningeal vasculitis associated with a compressive meningioma in a cat: a rare paraneoplastic syndrome

    Directory of Open Access Journals (Sweden)

    Les J. Gabor

    2012-02-01

    Full Text Available A 17 year old cat with a compressive meningioma was found to have an intradural, severe necrotizing vasculitis, spatially un associated with the neoplasm. Paraneoplastic vasculitis has been reported in two cases in the human literature associated with meningiomas. This is the first report of such an association in a domestic species.

  16. Diffuse Large B Cell Lymphoma in a Patient with Hypocomplementemic Urticarial Vasculitis

    Directory of Open Access Journals (Sweden)

    Calvo-Romero J

    2003-01-01

    Full Text Available Hypocomplementemic urticarial vasculitis (HUV is known to be associated with malignancies. Urticarial vasculitis has been linked to lymphomas, but to our knowledge, the association of HUV and non-Hodgkin lymphoma has not been described so far. A patient with HUV who developed 10 years later a diffuse large B cell lymphoma is reported here.

  17. NETosis: At the crossroads of rheumatoid arthritis, lupus, and vasculitis.

    Science.gov (United States)

    Berthelot, Jean-Marie; Le Goff, Benoit; Neel, Antoine; Maugars, Yves; Hamidou, Mohamed

    2017-05-01

    Suicidal NETosis differs from other mechanisms of cell death by the release of a lattice, composed of DNA associated with proteins citrullinated by protein-arginine deiminase 4, from neutrophils. These 'NETs' are composed of granule-derived proteins with microbicidal activity. A similar type of release occurs during vital NETosis, in which anuclear neutrophils maintain their chemotactic ability and imprison live bacteria, even after NET extrusion. Mitochondrial NETosis is limited to the expulsion of oxidised mitochondrial DNA and cytoplasmic enzymes. NETs include the targets of most autoantibodies found in rheumatoid arthritis, lupus, and vasculitis. The clinical and biological overlaps sometimes observed between bronchectiasis and RA, RA and SLE, or SLE and vasculitis, implicate NETosis as a major triggering event common to these disorders. NETosis increases the possibility of association between autoantigens and infectious antigens in mucosal biofilms, impairing the clearance of pathogens and possibly triggering autoimmune reactions. NETosis aggravates these three conditions and increases endothelial damage and the risk of thrombosis. However, the pathogenesis of RA, SLE, and vasculitis is not confined to autoantibodies against NET components, and other mechanisms have been suggested to explain the breakdown of tolerance to NET autoantigens, such as hypercitrullination. The question of whether continuous presentation of autoantigens mixed with antigens from dormant intracellular pathogens (released following suicidal, vital, or mitochondrial NETosis) is required to induce and sustain autoimmunity must be addressed. Inhibiting NETois may not be sufficient to improve autoimmune disorders whereas such latent infections remain uncontrolled. Copyright © 2016 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  18. Urticarial vasculitis in northern Spain: clinical study of 21 cases.

    Science.gov (United States)

    Loricera, Javier; Calvo-Río, Vanesa; Mata, Cristina; Ortiz-Sanjuán, Francisco; González-López, Marcos A; Alvarez, Lino; González-Vela, M Carmen; Armesto, Susana; Fernández-Llaca, Héctor; Rueda-Gotor, Javier; González-Gay, Miguel A; Blanco, Ricardo

    2014-01-01

    Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1-78 yr; interquartile range, 5-54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2-38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis is generally good

  19. Sudden bilateral sensorineural hearing loss associated with urticarial vasculitis.

    Science.gov (United States)

    Hall, A C; Leong, A C; Jiang, D; Fitzgerald-O'Connor, A

    2013-07-01

    Bilateral sensorineural hearing loss associated with recurrent urticarial skin lesions may be signs of underlying Muckle-Wells syndrome. Previous reports have described the hearing loss to be progressive in nature. To our knowledge, this paper presents the first published case of sudden onset, bilateral sensorineural hearing loss associated with urticarial vasculitis due to underlying Muckle-Wells syndrome. The patient underwent a cochlear implantation with a modest outcome. Cochlear implantation may help to rehabilitate sudden hearing loss associated with this condition, but early diagnosis may allow treatment with interleukin-1β inhibitors such as anakinra.

  20. Disseminated gonococcal infection presenting as vasculitis: a case report

    OpenAIRE

    Jain, Sangita; Win, Htet Nwe; Chalam, Venkat; Yee, Lian

    2007-01-01

    A 50‐year‐old man with alcoholic liver disease presented with fever, tenosynovitis, polyarthritis and a vasculitic rash on the hands and feet for 4 days. He had neutrophilia and raised inflammatory markers. He had no history of sore throat, urethral discharge or travel abroad. His initial blood cultures were negative, and he was treated for vasculitis with steroids. The rash and arthritis seemed to improve initially, but he had another episode of fever. Repeat blood cultures grew Neisseria go...

  1. Clinical significance of immunoglobulin deposition in leukocytoclastic vasculitis: a 5-year retrospective study of 88 patients at cleveland clinic.

    Science.gov (United States)

    Alalwani, Mohamed; Billings, Steven D; Gota, Carmen E

    2014-09-01

    To study the diagnostic utility and clinical associations of immunoglobulin deposition, determined by direct immunofluorescence (DIF) in cutaneous leukocytoclastic vasculitis (LCV). We performed a retrospective study of all biopsy-proven LCV cases seen at Cleveland Clinic between 2007 and 2012. All LCV cases in which DIF was performed were included. Of the 218 LCV cases, 106 cases had DIF performed and data from 88 cases were available: median (SD) age 53.3 (19.4), 52% male, 64.1% white, duration of rash 5.5 (20.8) months; follow-up 14 (19.7) months. DIF results showed any immunoglobulin and/or complement and/or fibrinogen in 70.5%, immunoglobulin A (IgA) in 36.4%, immunoglobulin M (IgM) in 21.6%, immunoglobulin G (IgG) in 11.4%. Patients with IgA deposition by DIF, compared with those without IgA, were younger, 44 (19) versus 56 (17) (P = 0.006), more likely to be white (P = 0.025) and had more organs affected by vasculitis (P = 0.002), higher incidence of gastrointestinal tract involvement (P = 0.0001) and renal disease (P = 0.006). No differences between rates of infection or malignancy were seen between DIF IgA, IgM, or IgG-positive versus negative patients. In patients with cutaneous LCV, IgA is the most common immunoglobulin found by DIF. IgA deposition, but not IgM or IgG, is predictive of associated renal and gastrointestinal organ involvement by vasculitis. No association between the type of immunoglobulin and preexisting infection or malignancy was found. DIF results add information that is clinically relevant to the diagnosis and management of LCV.

  2. Juvenile systemic lupus erythematosus and dermatomyositis associated with urticarial vasculitis syndrome: a unique presentation.

    Science.gov (United States)

    Macêdo, Patrícia A; Garcia, Carolina B; Schmitz, Monique K; Jales, Levi H; Pereira, Rosa M R; Carvalho, Jozélio F

    2012-11-01

    To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.

  3. EBNA1 expression in a lung transplant recipient with hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Berggren, Malin A M; Heinlen, Latisha; Isaksson, Asa; Nyström, Ulla; Ricksten, Anne

    2007-07-01

    This article describes a transplant recipient with underlying hypocomplementemic urticarial vasculitis syndrome who expressed persistently Epstein-Barr virus nuclear antigen 1 (EBNA1) in peripheral blood. The patient received a bilateral lung transplant and was subsequently followed with monitoring of EBV expression in peripheral blood. Evaluation of viral expression in peripheral blood, serum, and graft tissue was performed with RT-PCR, Q-PCR, indirect immunofluorescence, anti-peptide assays, and in situ hybridization; samples were collected at various time-points up to 91 days post-transplantation. The patient expressed EBNA1 in 8/10 (80%) of the peripheral blood samples tested during the post-transplantation period, and interestingly, even including the day of transplantation. After analyses of indicative EBV mRNA, EBNA1 expression was found mainly to be Qp-initiated EBNA1, known to be important for EBV maintenance. Anti-EBNA1 epitope mapping showed significantly higher and broader antibody responses to EBNA1 epitopes pre-transplantation when compared to normal controls and a matched lung transplant control. Post-transplantation this response was largely diminished but there were still epitopes significantly higher than controls. Our results show the presence of EBV-positive proliferating cells before onset of intensive immunosuppressive treatment. Although no previous connection between EBV and hypocomplementemic urticarial vasculitis syndrome has been reported, it is tempting to speculate that the continuous EBNA1 expression is not caused by immunosuppression or post-transplant lymphoproliferative disease, but may be a factor involved in the etiology of the autoimmune disease.

  4. 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.

  5. Are the 1990 American College of Rheumatology vasculitis classification criteria still valid?

    Science.gov (United States)

    Seeliger, Benjamin; Sznajd, Jan; Robson, Joanna C; Judge, Andrew; Craven, Anthea; Grayson, Peter C; Suppiah, Ravi S; Watts, Richard A; Merkel, Peter A; Luqmani, Raashid A

    2017-07-01

    Advances in diagnostic techniques have led to better distinction between types of vasculitis, potentially affecting the utility of the 1990 ACR classification criteria for vasculitis. This study tested the performance of these criteria in a contemporary vasculitis cohort. The Diagnosis and Classification in Vasculitis Study provided detailed clinical, serological, pathological and radiological data from patients with primary systemic vasculitis and clinical context-specific comparator conditions. Fulfilment of six ACR criteria sets and their diagnostic performance was evaluated in patients with a given type of vasculitis and its comparator conditions. Data from 1095 patients with primary systemic vasculitis and 415 with comparator conditions were available. For classification, sensitivities and specificities for ACR classification criteria were, respectively, 81.1% and 94.9% for GCA; 73.6% and 98.3% for Takayasu's arteritis; 65.6% and 88.7% for granulomatosis with polyangiitis; 57.0% and 99.8% for eosinophilic granulomatosis with polyangiitis; 40.6% and 87.8% for polyarteritis nodosa; 28.9% and 88.5% for microscopic polyangiitis; and 72.7% and 96.3% for IgA-vasculitis. Overall sensitivity was 67.1%. Of cases identified by their respective criteria, 16.9% also met criteria for other vasculitides. Diagnostic specificity ranged from 64.2 to 98.9%; overall, 113/415 comparators (27.2%) fulfilled at least one of the ACR classification criteria sets. Since publication of the ACR criteria for vasculitis, the sensitivity for each type of vasculitis, except GCA, has diminished, although the specificities have remained high, highlighting the need for updated classification criteria.

  6. Vessel Operator System

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Operator cards are required for any operator of a charter/party boat and or a commercial vessel (including carrier and processor vessels) issued a vessel permit from...

  7. Temporal concurrence of vasculitis and cancer: a report of 12 cases.

    Science.gov (United States)

    Hutson, T E; Hoffman, G S

    2000-12-01

    Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of temporal relationships, has led to skepticism about vasculitis being a paraneoplastic syndrome. The objective of the present study was to review cases of concurrent vasculitis and cancer at the Cleveland Clinic Foundation over an 18.5-year period and explore evidence that would support the notion of vasculitis being a type of paraneoplastic disease. Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using an ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients with known chronic autoimmune disease or serologic evidence of hepatitis B or C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of illness. During the 18.5 years of our study, more than 15 million inpatients and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patients had vasculitis independent of cancer, more than 69,000 patients had cancer, and 69 patients had been identified who had both malignancies and systemic vasculitis. Only 12 patients were identified in whom both vasculitis and cancer occurred within the same 12 months. Mean age was 65 years (range 45-79). There was no gender preference (M = F). In 8 of the 12 cases, diagnoses were made within 3 months of each other. In 6 of the patients, the diagnoses of both processes were made within 1 month. Ten of the 12 patients had vasculitis 1 to 3 months prior to or concurrent with the diagnosis of cancer. Six of the 12 patients had solid organ tumors, 4 had lymphoma, 1 had leukemia, and 1 had multiple myeloma. The most common vasculitis was cutaneous leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides included giant cell

  8. Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis

    Directory of Open Access Journals (Sweden)

    Senol Kobak

    2014-01-01

    Full Text Available A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later.

  9. DNASE1L3 mutations in hypocomplementemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Ozçakar, Z Birsin; Foster, Joseph; Diaz-Horta, Oscar; Kasapcopur, Ozgur; Fan, Yao-Shan; Yalçınkaya, Fatoş; Tekin, Mustafa

    2013-08-01

    Hypocomplementemic urticarial vasculitis syndrome (HUVS) is characterized by recurrent urticaria along with dermal vasculitis, arthritis, and glomerulonephritis. Systemic lupus erythematosus (SLE) develops in >50% of patients with HUVS, although the pathogenesis is unknown. The aim of this study was to identify the causative DNA mutations in 2 families with autosomal-recessive HUVS, in order to reveal the pathogenesis and facilitate the laboratory diagnosis. Autozygosity mapping was combined with whole-exome sequencing. In a family with 3 affected children, we identified a homozygous frameshift mutation, c.289_290delAC, in DNASE1L3. We subsequently identified another homozygous DNASE1L3 mutation leading to exon skipping, c.320+4delAGTA, in an unrelated family. The detected mutations led to loss of function, via either nonsense-mediated messenger RNA decay or abolished endonuclease activity, as demonstrated by a plasmid nicking assay. These results show that HUVS is caused by mutations in DNASE1L3, encoding an endonuclease that previously has been associated with SLE. Copyright © 2013 by the American College of Rheumatology.

  10. [Chromium-induced vasculitis-like purpuric allergic contact dermatitis].

    Science.gov (United States)

    Grange, A; Roth, B; Tortel, M-C; Guillaume, J-C

    2005-12-01

    Purpuric allergic contact dermatitis is a rare and poorly understood condition. A 27-year-old male patient with a personal history of atopic dermatitis since childhood consulted for chronic papular-purpuric rash present for 7 years. Moderate pruritus was seen. Profuse lesions were observed on the palms and soles and on the upper and lower limbs, with sparing of the trunk. These lesions consisted of purpuric papules, in some cases with crusts, forming large plaques. The clinical picture was initially suggestive of vasculitis, but this diagnosis was ruled out by histological examination and laboratory tests. Skin patch tests were evocative of chromium-induced contact dermatitis. Retrospective directed history-taking confirmed the relevance of the latter test since it revealed regular wearing of leather clothing. Lasting cure was achieved following eradication of the allergen. Reports of contact purpuric dermatitis are rare. This condition has been described principally for allergens consisting of rubber or dyes used in clothing. Our case was notable on account of the severity of the lesions, mimicking vasculitis, as well as the novelty of the incriminated allergen, chromium, found in leather garments. It underlines the value of routine skin patch tests in the event of chronic non-specific dermatitis. To our knowledge, this is the first reported case of chromium-induced purpuric allergic contact dermatitis.

  11. Leukocytoclastic vasculitis as early manifestation of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly.

    Science.gov (United States)

    Zoroquiain, Pablo; González, Sergio; Molgó, Montserrat; Rodríguez, Alejandra; Valbuena, José R

    2012-05-01

    Extensive necrotizing vasculitis (ENV) is a rare paraneoplastic phenomenon, and the majority of cases reported are associated with hematolymphoid neoplasms. Histologically, most cases of ENV represent leukocytoclastic vasculitis (LCV). Here we report the clinicopahological features of a 68-year-old man with ENV associated to a Epstein Barr virus-positive diffuse large B-cell lymphoma (EBV+DLBCL) of the elderly, a newly recognized lymphoproliferative disorder, most likely representing a paraneoplastic manifestation. The patient was treated with standard chemotherapy regimen for malignant lymphoma. Due to the extensive involvement of the extremities by ENV, surgical debridement was not feasible and a novel therapy based on CHITOSAN apposits was initiated with overall good response and subsequent re-epithelization of the skin lesions. The patient died of sepsis secondary to a Pseudomona pneumonia 17 months after diagnosis.

  12. 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.

  13. Caracterización clínica de las vasculitis cutáneas

    OpenAIRE

    Loricera García, Javier

    2015-01-01

    RESUMEN: El término de vasculitis cutánea (VC) comprende un conjunto de síndromes caracterizados por la inflamación de los vasos de la piel. Clínicamente se va a traducir fundamentalmente en una púrpura palpable e histológicamente en una vasculitis leucocitoclástica. La VC puede tratarse de un proceso primario o bien ser secundaria a una causa subyacente como vasculitis necrotizantes sistémicas, otras alteraciones del tejido conectivo, infecciones sistémicas o neoplasias. Una adecuada clasifi...

  14. Neonatal cerebral oxygenation is not linked to foetal vasculitis and predicts intraventricular haemorrhage in preterm infants

    DEFF Research Database (Denmark)

    Sørensen, Line Carøe; Maroun, Lisa Leth; Borch, K.

    2008-01-01

    AIM: The aim of the study was to compare the cerebral tissue oxygenation index (c-TOI) measured by near infrared spectroscopy (NIRS) in infants with and without foetal vasculitis. METHODS: Twenty-four infants with placental signs of a foetal inflammatory response (FIR), foetal vasculitis, were co.......002). CONCLUSION: Cerebral oxygenation was not affected in the first day of life in preterm infants born with foetal vasculitis, while cerebral oxygenation in infants that later developed intraventricular haemorrhage was impaired Udgivelsesdato: 2008/11...

  15. Omalizumab for Urticarial Vasculitis: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Misbah Nasheela Ghazanfar

    2015-01-01

    Full Text Available Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated with omalizumab.

  16. Omalizumab for Urticarial Vasculitis: Case Report and Review of the Literature.

    Science.gov (United States)

    Ghazanfar, Misbah Nasheela; Thomsen, Simon Francis

    2015-01-01

    Urticarial vasculitis is characterised by inflamed itching or burning red patches or wheals that resemble urticaria but persist for greater than 24 hours. It is often idiopathic but is sometimes associated with collagen-vascular disease, particularly systemic lupus erythematosus. Treatment options include oral antihistamines, oral corticosteroids, dapsone, colchicine or hydroxychloroquine. We describe a male patient with urticarial vasculitis who was treated with omalizumab (anti-IgE) with convincing results and provide a review of previous reports of patients with urticarial vasculitis treated with omalizumab.

  17. Outras vasculites pulmonares Other forms of pulmonary vasculitis

    Directory of Open Access Journals (Sweden)

    Carmen Sílvia Valente Barbas

    2005-07-01

    Full Text Available A poliangeíte microscópica, a arterite de Takayasu, a síndrome de Behçet, a púrpura de Henoch-Schönlein e as vasculites associadas às doenças do colágeno incluem-se entre as vasculites pulmonares. Seu diagnóstico é realizado associando-se as manifestações clínicas aos achados radiológicos e anatomopatológicos.Among the various forms of pulmonary vasculitis are microscopic polyangiitis, Takayasu's arteritis, Behçet's syndrome and Henoch-Schönlein purpura, as well as those forms related to rheumatologic diseases. The diagnosis is made through analysis of clinical manifestations, together with radiological and pathological findings.

  18. Circulating soluble adhesion molecules in ANCA-associated vasculitis.

    Science.gov (United States)

    Ara, J; Mirapeix, E; Arrizabalaga, P; Rodriguez, R; Ascaso, C; Abellana, R; Font, J; Darnell, A

    2001-02-01

    To evaluate whether changes in concentrations of soluble (s) E-selectin, sP-selectin, sL-selectin, intercellular adhesion molecule 1 (sICAM-1), and vascular cell adhesion molecule 1 (sVCAM-1) reflect disease activity in patients with ANCA-associated vasculitis and whether serum levels of these adhesion molecules are related to the degree of renal failure in patients with chronic renal failure (CRF). A sandwich ELISA was used to measure these soluble adhesion molecules in (i) sera from 20 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (10 patients with Wegener's granulomatosis (WG) and 10 patients with microscopic polyangiitis (MPA)), obtained at the time of diagnosis and during the remission period; (ii) sera from 40 patients with CRF not undergoing haemodialysis. At the time of diagnosis, serum levels of sE-selectin, sICAM-1 and sVCAM-1 (88+/-42 ng/ml, 437+/-184 ng/ml, 1720+/-1174 ng/ml respectively) were significantly higher in patients with ANCA-associated vasculitis than in healthy controls (P<0.0001, P=0.002 and P=0.001 respectively). Serum sP-selectin values did not differ from those obtained in normal donors. In contrast, sL-selectin levels (940+/-349 ng/ml) were significantly lower in patients than those recorded in healthy controls (P<0.0001). A significant decrease in concentrations of sE-selectin, sP-selectin, sICAM-1, and sVCAM-1 was observed between active and remission phases (P<0.0001, P=0.002, P=0.001 and P=0.001 respectively). No significant differences were observed in sL-selectin levels between active and remission phases. sL-selectin concentrations (802+/-306 ng/ml) during the remission phase remained lower than those observed in healthy controls (P<0.0001). No correlation was observed between serum creatinine and sE-selectin, sP-selectin, sICAM-1 and sVCAM-1 in patients of the CRF group. A slight negative correlation was established between creatinine and sL-selectin concentration. Increased serum levels of s

  19. Disseminated gonococcal infection presenting as vasculitis: a case report.

    Science.gov (United States)

    Jain, Sangita; Win, Htet Nwe; Chalam, Venkat; Yee, Lian

    2007-01-01

    A 50-year-old man with alcoholic liver disease presented with fever, tenosynovitis, polyarthritis and a vasculitic rash on the hands and feet for 4 days. He had neutrophilia and raised inflammatory markers. He had no history of sore throat, urethral discharge or travel abroad. His initial blood cultures were negative, and he was treated for vasculitis with steroids. The rash and arthritis seemed to improve initially, but he had another episode of fever. Repeat blood cultures grew Neisseria gonorrhoeae,and he received intravenous ceftriaxone followed by oral ciprofloxacin. He had marked improvement in rash, tenosynovitis and arthritis, and the fever dropped. He also had chlamydial urethritis and received azithromycin. The presentation of disseminated gonococcal infection after a presumptive episode of asymptomatic urethral gonorrhoea is highlighted.

  20. Kaposi′s sarcoma following immunosuppressive therapy for vasculitis

    Directory of Open Access Journals (Sweden)

    Tarik Bouattar

    2011-01-01

    Full Text Available Kaposi′s sarcoma (KS is widely reported to develop after renal transplantation and is induced by activation of a latent human herpes virus 8. We report the clinical features and outcome of a 50-year-old woman who presented with KS 18 weeks after starting immuno-suppressive therapy for vasculitis. She had positive-titer IgG antibody to human herpes virus 8. Cyclophosphamide pulses were interrupted, and prednisone was decreased gradually to 10 mg/day. Skin lesions showed important regression with stabilization of the general state and renal function. Eight months later, the patient presented with a diffuse cutaneous KS that required the discontinuation of steroids. Within 1 month, her general status and renal function deteriorated, and she died with a disseminated intravascular coagulation syndrome.

  1. Nomenclature and classification of vasculitis: lessons learned from granulomatosis with polyangiitis (Wegener's granulomatosis)

    Science.gov (United States)

    Jennette, J C

    2011-01-01

    Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate a name may be, if it is not usable and used it is of no lasting value. This brief commentary focuses on the nomenclature of systemic vasculitides, and uses as a prime example Wegener's granulomatosis, which has been renamed recently ‘granulomatosis with polyangiitis’, in part because of concerns about the suitability of Friedrich Wegener as the source of an eponym. The most distinctive pathological feature of Wegener's granulomatosis is multi-focal necrotizing inflammation that has long been called granulomatosis. The systemic variant of Wegener's granulomatosis also is characterized by inflammation in many different vessels or different types, i.e. polyangiitis. Thus, granulomatosis with polyangiitis is a very appropriate alternative term for Wegener's granulomatosis. This term also is in accord with the name for a closely related vasculitis, i.e. microscopic polyangiitis. Terms that indicate aetiology and pathogenesis, when known, are useful to include in names for diseases (diagnoses). Anti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) are implicated in the cause of granulomatosis with polyangiitis and thus also should be specified in the diagnosis (e.g. PR3-ANCA-positive granulomatosis with polyangiitis or MPO-ANCA-positive microscopic polyangiitis). As our understanding of the clinical manifestations, pathogenesis and aetiology of vasculitides change over time, the names and approaches for diagnosing these diseases will change accordingly. PMID:21447122

  2. ANCA-associated vasculitis: a comparison of cases presenting to nephrology and rheumatology services.

    Science.gov (United States)

    McNicholas, B A; Griffin, T P; Donnellan, S; Ryan, L; Garrahy, A; Coughlan, R; Giblin, L; Lappin, D; Reddan, D; Carey, J J; Griffin, M D

    2016-12-01

    Anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitis (AAV) is a disease characterized by inflammation of small vessels and detectable ANCA in the circulation. Patients may develop a broad spectrum of clinical features ranging from indolent sino-nasal disease and rashes to fulminant renal failure or acute life-threatening pulmonary haemorrhage. Consequently, patients with AAV present to a variety of specialties including nephrology and rheumatology, whose training and approaches to management of such patients may differ. There is little literature comparing patients presenting to different specialties and their outcomes. We compared two cohorts of patients with ANCA-positive AAV presenting to either the rheumatology or nephrology department at Galway University Hospitals from June 2002 to July 2011. A standardized data collection form was used to collect information regarding baseline demographics, manifestations of AAV, initial management, relapses and complications. Forty-five patients were included in this study (15 rheumatology/30 nephrology). The nephrology cohort was older, had a higher C-reactive protein, Birmingham Vascular Activity Score and ANCA titer at presentation compared to the rheumatology group. Induction treatment varied between the cohorts with rheumatology patients most commonly receiving a combination of oral corticosteroids (73%) and methotrexate (60%) and nephrology patients receiving a combination of intravenous corticosteroids (93%) and cyclophosphamide (90%). Fifty-three percent of the rheumatology patients who completed induction therapy relapsed compared to 30% of the nephrology patients. This study presents two different cohorts of patients with the same disease that were managed by two different disciplines. It highlights the heterogeneity of AAV and the importance of interdisciplinary communication and cooperation when managing these patients. © The Author 2016. Published by Oxford University Press on behalf of the

  3. BIOASSAY VESSEL FAILURE ANALYSIS

    Energy Technology Data Exchange (ETDEWEB)

    Vormelker, P

    2008-09-22

    Two high-pressure bioassay vessels failed at the Savannah River Site during a microwave heating process for biosample testing. Improper installation of the thermal shield in the first failure caused the vessel to burst during microwave heating. The second vessel failure is attributed to overpressurization during a test run. Vessel failure appeared to initiate in the mold parting line, the thinnest cross-section of the octagonal vessel. No material flaws were found in the vessel that would impair its structural performance. Content weight should be minimized to reduce operating temperature and pressure. Outer vessel life is dependent on actual temperature exposure. Since thermal aging of the vessels can be detrimental to their performance, it was recommended that the vessels be used for a limited number of cycles to be determined by additional testing.

  4. F-18-fluorodeoxyglucose positron emission tomography in diagnosis and follow-up of patients with different types of vasculitis.

    Science.gov (United States)

    Bleeker-Rovers, C P; Bredie, S J H; van der Meer, J W M; Corstens, F H M; Oyen, W J G

    2003-10-01

    F-18-fluorodeoxyglucose (FDG) accumulates in inflammatory cells due to an increased metabolic rate. Therefore, FDG positron emission tomography (PET) represents a promising imaging technique in patients with vasculitis. The aim of this study was to assess the value of FDG PET in the diagnosis of different types of vasculitis. The results of FDG PET performed because of suspected vasculitis or fever of unknown origin with results indicating vasculitis were reviewed. These results were compared with the final diagnosis, based on the American College of Rheumatology 1990 criteria. FDG PET was ordered because of suspected vasculitis in 20 patients, because of fever of unknown origin in two patients, and for follow-up of vasculitis in five patients. Fourteen patients were diagnosed with vasculitis (giant cell arteritis n = 5, polymyalgia rheumatica n = 2, polyarteritis nodosa n = 3, Takayasu n = 1, Churge-Strauss n = 1, Wegener's granulomatosis n = 1, vasculitis skin n = 1), two patients were diagnosed with fibromuscular dysplasia and one patient had media necrosis of the aorta. In five patients no diagnosis could be reached. FDG PET results were considered to be true-positive in ten patients, true-negative in 14 patients and false-negative in three patients resulting in a positive predictive value of 100% and a negative predictive value of 82%. FDG PET appears to be a promising new imaging technique in diagnosing and determining the extent of various forms of vasculitis. Furthermore, FDG PET may become a useful tool for evaluating the effect of treatment of vasculitis.

  5. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis : 2-year results of a randomised trial

    NARCIS (Netherlands)

    Jones, Rachel B.; Furuta, Shunsuke; Tervaert, Jan Willem Cohen; Hauser, Thomas; Luqmani, Raashid; Morgan, Matthew D.; Peh, Chen Au; Savage, Caroline O.; Segelmark, Marten; Tesar, Vladimir; van Paassen, Pieter; Walsh, Michael; Westman, Kerstin; Jayne, David R. W.; Stegeman, C. A.

    Objectives The RITUXVAS trial reported similar remission induction rates and safety between rituximab and cyclophosphamide based regimens for antineutrophil cytoplasm antibody (ANCA)-associated vasculitis at 12months; however, immunosuppression maintenance requirements and longer-term outcomes after

  6. Key advances in the clinical approach to ANCA-associated vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    The updated nomenclature for vasculitis defines this varied group of disorders by aetiology, specific features of pathogenesis and clinical symptoms; diagnostic and classification criteria for clinical practice are in development. Here, I review some important advances in the management of

  7. Is serum HMGB1 a biomarker in ANCA-associated vasculitis?

    NARCIS (Netherlands)

    de Souza, Alexandre W.S.; Westra, Johanna; Bijzet, Johan; Limburg, Pieter C.; Stegeman, Coen A.; Bijl, Marc; Kallenberg, Cees G.M.

    2013-01-01

    Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are systemic inflammatory disorders that include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), Churg-Strauss syndrome and renal limited vasculitis (RLV). Extracellular high-mobility group

  8. Pathogenesis of ANCA-associated vasculitis : recent insights from animal models

    NARCIS (Netherlands)

    van Timmeren, Mirjan M.; Heeringa, Peter

    Purpose of review To provide an update on animal models of antineutrophil cytoplasmic autoantibody (ANCA)-mediated vasculitis and highlight recent insights gained from studies in these models pertaining to immunopathogenesis. Recent findings Animal models support the pathogenic potential of

  9. Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia.

    Science.gov (United States)

    Pinto-Almeida, Teresa; Caetano, Mónica; Alves, Rosário; Selores, Manuela

    2013-01-01

    Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

  10. Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia*

    Science.gov (United States)

    Pinto-Almeida, Teresa; Caetano, Mónica; Alves, Rosário; Selores, Manuela

    2013-01-01

    Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed. PMID:24474109

  11. Tumor necrosis factor-like weak inducer of apoptosis and its receptor fibroblast growth factor-inducible 14 are expressed in urticarial vasculitis.

    Science.gov (United States)

    Li, Mengmeng; Chen, Tao; Guo, Zaipei; Li, Jingyi; Cao, Na

    2013-11-01

    Tumor necrosis factor (TNF)-like weak inducer of apoptosis (TWEAK), a member of the TNF family, has been implicated as a pro-inflammatory cytokine in many types of autoimmune and infectious diseases. However, information about TWEAK in dermatological diseases is limited. To date, no studies have investigated the roles of TWEAK in patients with urticarial vasculitis (UV). This study aimed to assess serum TWEAK levels, together with TWEAK and fibroblast growth factor-inducible 14 (Fn14) expressions of skin lesions in patients with UV. Serum TWEAK levels in patients with UV, together with patients with cutaneous leukocytoclastic angiitis (CLA) and healthy controls were detected by enzyme-linked immunosorbent assay; TWEAK and Fn14 expressions of skin lesions were analyzed by immunohistochemistry. Results showed that TWEAK and Fn14 were abundantly expressed in the dermal vessel wall of lesional skin in patients with UV but not healthy controls. Serum TWEAK levels in the acute stage in patients with UV were significantly higher than those in the convalescent stage and healthy controls. Serum TWEAK levels were elevated significantly in patients with CLA compared with those in healthy controls. Our previous study indicated that TWEAK may be an important mediator for the development of vascular inflammation in skin. In addition, we also found that TWEAK blockade substantially reduced vascular damage and perivascular leukocyte infiltrates in lipopolysaccharide-induced cutaneous vasculitis. Our study shows that TWEAK may be associated with the pathogenesis of UV; it is therefore suggested that TWEAK may be a potential therapeutic target for UV and other types of cutaneous vasculitis. © 2013 Japanese Dermatological Association.

  12. Vasculitis secundaria a infección por Fasciola hepática Secondary vasculitis to infection by Fasciola hepatica

    Directory of Open Access Journals (Sweden)

    Germán Málaga

    2012-09-01

    Full Text Available Se presenta el caso de un paciente varón de 38 años, procedente de una zona endémica para fascioliasis hepática en el Perú. Fue hospitalizado por presentar un cuadro de cuatro semanas de evolución; con fiebre, mialgias intensas, lesiones eritematosas y dolorosas en las regiones de extensión de las extremidades. La electromiografía y la velocidad de conducción nerviosa mostraron una miopatía inflamatoria global. La biopsia de piel evidenció una vasculitis de tipo poliarteritis nodosa. En el proceso de evaluación previa a la terapia inmunosupresora, se hallaron huevos de Fasciola hepática en el examen coproparasitológico. El diagnóstico de fascioliasis se confirmó con fas2-ELISA: 0,46 (VN There is a case of a 38 year-old male patient coming from an area where hepatic fascioliasis is endemic in Peru. He was hospitalized because he showed 4 weeks of symptoms like fever, intense myalgias, erythematous and painful injuries on limb extensions. The electromyography and nerve conduction velocity showed a global inflammatory myopathy. A skin biopsy showed polyarteritis nodosa-type vasculitis. During the evaluation process prior to the immunosuppressive therapy, hepatic Fasciola eggs were found in the parasitological examination of stools. The fascioliasis diagnosis was confirmed by fas2-ELISA: 0.46 (VN <0.20. Clinical symptoms started to subside after treatment with ticlabendazol. Contact with the patient was maintained for a year and there was no evidence of disease recurrence, and he was asymptomatic

  13. Decline in rheumatoid vasculitis prevalence among US veterans: a retrospective cross-sectional study.

    Science.gov (United States)

    Bartels, Christie; Bell, Carolyn; Rosenthal, Ann; Shinki, Kazuhiko; Bridges, Alan

    2009-09-01

    To examine trends in the prevalence of rheumatoid vasculitis in a national US population comprising both hospitalized and ambulatory patients with rheumatoid arthritis (RA). In this serial cross-sectional study, we analyzed data on hospitalized and ambulatory patients spanning 22 years (1985-2006) and 10 years (1997-2006), respectively, to determine the prevalence of rheumatoid vasculitis, as defined by the International Classification of Diseases, Ninth Revision. Our search encompassed data collected on a predominantly male study population during 10 million hospitalizations and outpatient visits, and included annual data on >37,000 RA patients. To test for a decrease in rheumatoid vasculitis prevalence, breakpoint analysis was performed using stepwise Chow and Durbin-Watson tests. There was a clear decline in the prevalence of rheumatoid vasculitis, and this decline remained evident even after accounting for a decreased number of hospitalizations among RA patients. Peak prevalence occurred among hospitalized patients in the 1980s, and prevalence gradually declined throughout the 1990s. Furthermore, simultaneous breakpoints representing a significant drop in rheumatoid vasculitis prevalence between the years 2000 and 2001 were demonstrated for both inpatients (P < 0.000) and outpatients (P < 0.003). The prevalence of vasculitis dropped 53% among inpatients and 31% among outpatients between 2000 and 2001. Our results demonstrate a significant decline in rheumatoid vasculitis prevalence after 2000 in this nationwide sample of hospitalized and ambulatory patients. The clear, consistent drop in prevalence provides an opportunity for the formulation of causal hypotheses, including consideration of the impact of biologic agents used to treat RA, on rheumatoid vasculitis.

  14. BPC 157 and blood vessels.

    Science.gov (United States)

    Seiwerth, Sven; Brcic, Luka; Vuletic, Lovorka Batelja; Kolenc, Danijela; Aralica, Gorana; Misic, Marija; Zenko, Anita; Drmic, Domagoj; Rucman, Rudolf; Sikiric, Predrag

    2014-01-01

    This review focuses on the described effects of BPC 157 on blood vessels after different types of damage, and elucidate by investigating different aspects of vascular response to injury (endothelium damage, clotting, thrombosis, vasoconstriction, vasodilatation, vasculoneogenesis and edema formation) especially in connection to the healing processes. In this respect, BPC 157 was concluded to be the most potent angiomodulatory agent, acting through different vasoactive pathways and systems (e.g. NO, VEGF, FAK) and leading to optimization of the vascular response followed, as it has to be expected, by optimization of the healing process. Formation of new blood vessels involves two main, partly overlapping mechanisms, angiogenesis and vasculogenesis. The additional mechanism of arteriogenesis is involved in the formation of collaterals. In conjunction with blood vessel function, we at least have to consider leakage of fluid/proteins/plasma, resulting in edema/exudate formation as well as thrombogenesis. Blood vessels are also strongly involved in tumor biology. In this aspect, we have neoangiogenesis resulting in pathological vascularization, vascular invasion resulting in release of metastatic cells and the phenomenon of homing resulting in formation of secondary tumors--metastases.

  15. Membranoproliferative glomerulonephritis presenting as arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis: a case report.

    Science.gov (United States)

    Park, Chuiyoung; Choi, Seung Won; Kim, Misung; Park, Jongha; Lee, Jong Soo; Chung, Hyun Chul

    2014-10-22

    Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud's arthropathy, and valvular heart disease have been reported. A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy.

  16. Minocycline-induced polyarteritis nodosa-like vasculitis presenting as brainstem stroke.

    Science.gov (United States)

    Klaas, James P; Matzke, Thomas; Makol, Ashima; Fulgham, Jimmy R

    2015-05-01

    Minocycline use has been associated with the development of autoimmune disorders, including drug-induced vasculitis. Previously published reports suggest that clinical manifestations are limited to cutaneous, constitutional, or musculoskeletal symptoms. To our knowledge there has been only one reported patient with ischemic stroke in the setting of minocycline-induced vasculitis. We describe a 26-year-old woman, with no vascular risk factors, who had an ischemic pontine stroke in the setting of biopsy-proven minocycline-induced polyarteritis nodosa-like vasculitis. Discontinuation of minocycline resulted in resolution of the vasculitis, and she has not had any recurrent ischemic events. This report shows that ischemic strokes may occur as a result of minocycline-induced vasculitis. While this is likely a rare association, recognition is important given the widespread use of minocycline and the potential for devastating consequences in a young population. Consequently, drug-induced vasculitis should be considered in patients with an ischemic stroke taking minocycline. Copyright © 2014 Elsevier Ltd. All rights reserved.

  17. Guam Abandoned Vessel Inventory

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Guam. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  18. Florida Abandoned Vessel Inventory

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Florida. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral...

  19. Vessel Arrival Info - Legacy

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Vessel Arrival Info is a spreadsheet that gets filled out during the initial stage of the debriefing process by the debriefer. It contains vessel name, trip...

  20. Mechanosensing in developing lymphatic vessels.

    Science.gov (United States)

    Planas-Paz, Lara; Lammert, Eckhard

    2014-01-01

    The lymphatic vasculature is responsible for fluid homeostasis, transport of immune cells, inflammatory molecules, and dietary lipids. It is composed of a network of lymphatic capillaries that drain into collecting lymphatic vessels and ultimately bring fluid back to the blood circulation. Lymphatic endothelial cells (LECs) that line lymphatic capillaries present loose overlapping intercellular junctions and anchoring filaments that support fluid drainage. When interstitial fluid accumulates within tissues, the extracellular matrix (ECM) swells and pulls the anchoring filaments. This results in opening of the LEC junctions and permits interstitial fluid uptake. The absorbed fluid is then transported within collecting lymphatic vessels, which exhibit intraluminal valves that prevent lymph backflow and smooth muscle cells that sequentially contract to propel lymph.Mechanotransduction involves translation of mechanical stimuli into biological responses. LECs have been shown to sense and respond to changes in ECM stiffness, fluid pressure-induced cell stretch, and fluid flow-induced shear stress. How these signals influence LEC function and lymphatic vessel growth can be investigated by using different mechanotransduction assays in vitro and to some extent in vivo.In this chapter, we will focus on the mechanical forces that regulate lymphatic vessel expansion during embryonic development and possibly secondary lymphedema. In mouse embryos, it has been recently shown that the amount of interstitial fluid determines the extent of lymphatic vessel expansion via a mechanosensory complex formed by β1 integrin and vascular endothelial growth factor receptor-3 (VEGFR3). This model might as well apply to secondary lymphedema.

  1. 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.

  2. 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

  3. ALICE HMPID Radiator Vessel

    CERN Multimedia

    2003-01-01

    View of the radiator vessels of the ALICE/HMPID mounted on the support frame. Each HMPID module is equipped with 3 indipendent radiator vessels made out of neoceram and fused silica (quartz) windows glued together. The spacers inside the vessel are needed to stand the hydrostatic pressure. http://alice-hmpid.web.cern.ch/alice-hmpid

  4. Leukocytoclastic vasculitis in an adolescent with ulcerative colitis: Report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    G Tyler Butts

    2014-08-01

    Full Text Available An adolescent female with long-standing, difficult-to-control ulcerative colitis developed leukocytoclastic vasculitis, a rare cutaneous extra-intestinal manifestation of the inflammatory bowel disease. The authors provide a literature review on leukocytoclastic vasculitis complicating ulcerative colitis. Furthermore, the clinical features of leukocytoclastic vasculitis are compared and contrasted with the more common cutaneous extra-intestinal manifestations of inflammatory bowel disease, erythema nodosum, and pyoderma gangrenosum.

  5. Clinical analysis of ANCA-associated renal vasculitis patients with chronic dialysis.

    Science.gov (United States)

    Chen, Yong-Xi; Zhang, Wen; Chen, Xiao-Nong; Ni, Li-Yan; Shen, Ping-Yan; Wang, Wei-Ming; Chen, Nan

    2014-01-01

    Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitute a subgroup of life-threatening diseases which affects the kidney in more than half of the patients at diagnosis. Currently, little has been published focusing on AAV patients with dialysis. We analysed AAV patients with chronic dialysis to provide more detailed information. From 1997 to 2011, AAV patients complicated by renal involvement resulting in end-stage renal disease (ESRD) and had undergone haemodialysis (HD) or peritoneal dialysis (PD) for at least 3 months in Shanghai Ruijin hospital were retrospectively analysed in this study. Their data were also compared to those without dialysis at the same time. We enrolled 49 AAV patients with chronic dialysis. 41 required dialysis at initial presentation and rest 8 progressed to ESRD during follow-up. 19 HD patients died and 6 PD patients died during follow-up, and infection was the most common cause among the patients. There was no significant difference regarding survival between HD patients and PD patients (p>0.05). However anaemia and level of triglyceride was more significantly improved in HD patients at the end of observation (pdialysis dependency, dialysis patients presented higher percentage of hypertension (pdialysis patients (pdialysis dependence. Our study suggests that haemodialysis and peritoneal dialysis are two comparable dialysis modalities for AAV patients with ESRD. However, AAV patients with dialysis dependency had worse outcome in comparison with those without dialysis.

  6. Profile of retinal vasculitis in a tertiary eye care center in Eastern India

    Directory of Open Access Journals (Sweden)

    Kumar Saurabh

    2011-01-01

    Full Text Available Aims: To provide a fact file on the etiology, clinical presentations and management of retinal vasculitis in Eastern India. Materials and Methods: Retrospective, record based analysis of retinal vasculitis cases in a tertiary care center in Eastern India from January 2007 to December 2009 . Results: One hundred and thirteen eyes of 70 patients of retinal vasculitis were included in this study. Sixty (85.7% patients were male (mean age 33± 11.1 years and 10 (14.3% were female (mean age 32.4 ± 13.6 years. Vasculitis was bilateral in 43 (61.4% and unilateral in 27 (38.6% patients. Commonest symptoms were dimness of vision (73; 64.6% and floaters (36; 31.9%. Vascular sheathing (82; 72.6% and vitritis (51; 45.1% were commonest signs. Mantoux test was positive in 21 (30% patients but tuberculosis was confirmed in only four (5.71% patients. Raised serum angiotensin-converting enzyme level and positive antinuclear antibody level were reported in four (5.71% patients each. Human leukocyte antigen B5 (HLA B5 marker was present in one (1.4% patient. However, none of the total 70 patients were found to have a conclusively proven systemic disease attributable as the cause of retinal vasculitis. Oral corticosteroid (60; 85.7% was the mainstay of treatment. Forty-eight (42.5% eyes maintained their initial visual acuity and 43 (38% gained one or more line at mean follow-up of 16.6± 6.3 months. Conclusion: Retinal vasculitis cases had similar clinical presentations and common treatment plan. There was no systemic disease association with vasculitis warranting a careful approach in prescribing investigations.

  7. Leukocytoclastic vasculitis: A window to systemic Churg Strauss syndrome

    Directory of Open Access Journals (Sweden)

    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.

  8. Florid urticarial vasculitis heralding a flare up of ulcerative colitis.

    Science.gov (United States)

    Boules, Evon; Lyon, Calum

    2014-12-22

    A 75-year-old man with ulcerative colitis (UC) and diet controlled diabetes mellitus presented with a 3-week history of slightly itchy, red plaques on both lower limbs ascending gradually to cover the trunk and arms. One week later, he developed a flare up of his UC. Routine blood tests showed modest drop in haemoglobin (122 g/L) and C reactive protein (85 mg/L). Serology was remarkable for high antiproteinase 3 (c-ANCA). Serum electrophoresis showed a mildly positive paraprotein band (γ region). Stool culture was negative. Urine analysis showed proteinuria. Skin biopsy showed features of urticarial vasculitis (UV). He underwent a flexible sigmoidoscopy after the flare up showed mildly active UC. The patient was given hydrocortisone for 7 days and then prednisolone. Both rash and UC subsided. Electrophoresis was repeated 4 weeks later showing normal pattern. Prednisolone has been gradually reduced. Although rare, UV can be considered as one of the skin manifestations of UC. 2014 BMJ Publishing Group Ltd.

  9. Normocomplementaemic Urticarial Vasculitis in a 19-Month-Old Girl

    Science.gov (United States)

    2016-01-01

    Urticaria is common in children. Urticarial vasculitis (UV) is a potentially more serious, rare variant. The youngest reported case was 12 months of age. A systemically well, 19-month-old girl presented with her mother who was concerned about the development of a rash. On presentation, the child had normal vital signs, was alert, and was well and playing with toys. There was a widespread urticarial rash (raised, pruritic, and erythematous) that was most apparent on the trunk with minimal rash on the legs. Overlying this urticarial rash in a similar distribution was a blotchy, palpable purpuric rash and associated hyperpigmentation. Investigations revealed a normal level of haemoglobin, white cells, platelets, and electrolytes. Renal function, international normalised ratio, and activated partial thromboplastin time were all normal. There was no blood or protein in the urine. The erythrocyte sedimentation rate was mildly elevated at 19 mm/hour. Complement results (including C1q) obtained later were normal. This case is striking not only because of the rarity of UV in children but also due to the unique diagnostic and prognostic challenges that it raises. PMID:27818822

  10. Normocomplementaemic Urticarial Vasculitis in a 19-Month-Old Girl

    Directory of Open Access Journals (Sweden)

    Peter Williams

    2016-01-01

    Full Text Available Urticaria is common in children. Urticarial vasculitis (UV is a potentially more serious, rare variant. The youngest reported case was 12 months of age. A systemically well, 19-month-old girl presented with her mother who was concerned about the development of a rash. On presentation, the child had normal vital signs, was alert, and was well and playing with toys. There was a widespread urticarial rash (raised, pruritic, and erythematous that was most apparent on the trunk with minimal rash on the legs. Overlying this urticarial rash in a similar distribution was a blotchy, palpable purpuric rash and associated hyperpigmentation. Investigations revealed a normal level of haemoglobin, white cells, platelets, and electrolytes. Renal function, international normalised ratio, and activated partial thromboplastin time were all normal. There was no blood or protein in the urine. The erythrocyte sedimentation rate was mildly elevated at 19 mm/hour. Complement results (including C1q obtained later were normal. This case is striking not only because of the rarity of UV in children but also due to the unique diagnostic and prognostic challenges that it raises.

  11. A case of aortic and mitral valve involvement in granulomatosis with polyangiitis.

    Science.gov (United States)

    Espitia, Olivier; Droy, Laure; Pattier, Sabine; Naudin, Frédérique; Mugniot, Antoine; Cavailles, Arnaud; Hamidou, Mohamed; Bruneval, Patrick; Agard, Christian; Toquet, Claire

    2014-01-01

    Granulomatosis with polyangiitis (GPA) (Wegener's) is a necrotizing systemic vasculitis of the small-sized blood vessels, affecting kidneys, lungs, upper respiratory tract and skin. Cardiac valvular involvement is an uncommon manifestation of GPA. We report the case of a 60-year-old woman with arthritis and lung nodules due to GPA without antineutrophil cytoplasmic antibodies (ANCA) at time of diagnosis. Remission was obtained with cyclophosphamide and corticosteroid. Azathioprine was then prescribed for 2years. Four years later, she developed severe inflammatory aortic and mitral valvular involvement characterized by GPA typical histopathological valvular lesions. Search for ANCA was positive at this time (anti-myeloperoxidase). Cardiac valvular involvement is a rare and potentially fatal complication of GPA and may misleadingly suggest infectious endocarditis. A review of literature revealed few cases of histologically well-documented cardiac valvular involvement in GPA. Pathologists should be aware of valvular heart diseases in GPA, which usually comprise valvular necrotic lesions without any microbial agents. Copyright © 2014 Elsevier Inc. All rights reserved.

  12. Cardiac involvement in Wegener granulomatosis diagnosed at autopsy.

    Science.gov (United States)

    Mukhopadhyay, Sanjay; Hensley, Richard G; Tazelaar, Henry D

    2010-01-01

    Wegener granulomatosis (WG) is a systemic vasculitis classically involving the lungs, kidneys, and upper respiratory tract. Involvement of other sites does occur but is less frequent. Clinically evident cardiac involvement is uncommon. There are only a few cases in the literature with documentation of the histologic appearance of cardiac involvement in WG. We report a case of a previously healthy 37-year-old man who presented with a one-week history of cough and weakness and a one-day history of shortness of breath. At presentation, he was hypoxic and required intubation. Upon hospitalization, he deteriorated rapidly, became bradycardic and expired the same night. Infection was suspected clinically as the cause of illness and death. At autopsy, the lungs showed diffuse alveolar hemorrhage with capillaritis, diffuse alveolar damage, and parenchymal necrosis with necrotizing granulomas. The heart was enlarged and showed a mixed inflammatory infiltrate composed of neutrophils, eosinophils, and histiocytes, with focal myocyte necrosis. Granulomas, giant cells, and vasculitis were absent. The esophagus showed submucosal vasculitis. A diagnosis of WG was made. Postmortem serology for c-ANCA was positive, and all cultures were negative, confirming the diagnosis. Cardiac involvement is an underrecognized and potentially fatal complication of WG. The histologic findings in the heart may consist of a non-specific inflammatory infiltrate without granulomas or vasculitis, raising a wide differential diagnosis. Copyright 2010 Elsevier Inc. All rights reserved.

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

    Directory of Open Access Journals (Sweden)

    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.

  14. Policondritis recidivante y compromiso neurológico Neurologic involvement in relapsing polychondritis

    Directory of Open Access Journals (Sweden)

    María A. Requena

    2007-04-01

    Full Text Available La policondritis recidivante (PR es un trastorno autoinmune multisistémico, de etiología desconocida, que se caracteriza por episodios recurrentes de inflamación y destrucción progresiva de las estructuras cartilaginosas y el tejido conectivo, pero que también puede afectar las estructuras con proteinglicanos como ojos, corazón, riñón y vasos sanguíneos produciendo vasculitis. Se presenta el caso de un paciente con criterios clínicos de policondritis recidivante con convulsiones, y una resonancia nuclear magnética de cerebro con imágenes hiperintensas en T2 y FLAIR, multifocales tanto en la sustancia gris como blanca, sugerentes de lesiones isquémicas, que mejoró con dosis altas de glucocorticoides. Se señala en esta enfermedad de muy baja prevalencia la presentación inusual de manifestaciones neurológicas reversibles con tratamiento.Relapsing polychondritis is a multysistemic inflammatory disease, of unknown etiology. It is characterized by recurrent episodes of inflammation with progressive destruction of cartilaginous structures and connective tissue. It may also affect tissues that contain proteoglycans like eyes, heart, kidney, and blood vessels causing a picture of vasculitis. We report here the case of a patient with relapsing polychondritis and seizures. The magnetic resonance imaging of the brain showed multiple hyperintense signals in the T2 weighted images and fluid-attenuated inversion recovery (flair, suggesting ischemic injuries, that improved with high doses of glucocorticoids. We remark the unusual presentation with neurological involvement in this uncommon disease that has improved with treatment.

  15. The Pharmacogenomic Association of Fc gamma Receptors and Cytochrome P450 Enzymes With Response to Rituximab or Cyclophosphamide Treatment in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Cartin-Ceba, Rodrigo; Indrakanti, Divya; Specks, Ulrich; Stone, John H.; Hoffman, Gary S.; Kallenberg, Cees G. M.; Langford, Carol A.; Merkel, Peter A.; Spiera, Robert F.; Monach, Paul A.; St. Clair, E. William; Seo, Philip; Tchao, Nadia K.; Ytterberg, Steven R.; Brunetta, Paul G.; Song, Huijuan; Birmingham, Dan; Rovin, Brad H.; Grp, RAVE-Immune Tolerance Network Res

    Objective The Rituximab in ANCA-Associated Vasculitis (RAVE) trial compared rituximab to cyclophosphamide as induction therapy for the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. We undertook the current study to determine whether known single-nucleotide

  16. In-vessel core debris retention through external flooding of the reactor pressure vessel. State-of-the-art report

    Energy Technology Data Exchange (ETDEWEB)

    Heel, A.M.J.M. van

    1995-07-01

    An overview of the state-of-the-art knowledge on the ex-vessel flooding accident management strategy for severe accidents in a NPP has been given. The feasibility has been discussed, as well as the in- and ex-vessel phenomena, which influence the structural integrity of the vessel. Finally, some computer codes with the ability to model the phenomena involved in ex-vessel flooding have been discussed. (orig./HP).

  17. Peripheral nervous system involvement in HCV-related mixed cryoglobulinemia

    Directory of Open Access Journals (Sweden)

    F. Bravaccio

    2011-09-01

    Full Text Available In HCV-related mixed cryoglobulinemia (MC a peripheral neuropathy (PN may occur. To evaluate the prevalence and the characteristics of PN, 133 consecutive patients with HCV-MC (117 type II, 16 type III were studied. Neurologic evaluation was performed according to the guidelines of Italian Group for the Study of Cryoglobulinemias, using a neurological disability score and a neurological symptom score. In 52/133 patients an electrophysiologic study (ENG of ulnar, peroneal and sural nerves was performed. For 27/52 patients ENG data registered at different times (interval 12-96 months were available. In 11 patients a sural nerve biopsy was obtained. An overt PN, mostly as sensory asymmetrical or symmetrical nerve impairement, was found in 107/133 patients (80.4%. ENG abnormalities-reduction or absence of sensory and sometimes of motor action potential, normal or slightly impaired nerve conduction velocity, consistent with axonal damage- were detected in 48/52 patients (92.3%. In 26 out of the 27 patients observed at different times an evolution of PN was found. Nerve biopsies showed a prevalent axonal damage, swollen endotelial cells in epi- and perineurial vessels and scarce mononuclear perivascular infiltrates. No leukocytoclastic vasculitis was observed. Immunoglobulins and complement in sub-perineurial vessel wall were detected. Conclusions. In HCV-MC a PN is frequent. It is mostly a sensory and progressively worsenig axonopathy. Different mechanisms may be involved in the pathogenesis of this disorder and a direct role of HCV cannot be excluded.

  18. Transient evolution of inter vessel gap pressure due to relative thermal expansion between two vessels

    Science.gov (United States)

    Natesan, K.; Selvaraj, P.; Chellapandi, P.; Chetal, S. C.

    2002-08-01

    In a typical liquid metal cooled fast breeder reactor (LMFBR), a cylindrical sodium filled main vessel, which carries the internals such as reactor core, pumps, intermediate heat exchangers etc. is surrounded by another vessel called safety vessel. The inter vessel gap is filled with nitrogen. During a thermal transient in the pool sodium, because of the relative delay involved in the thermal diffusion between MV and SV, they are subjected to relative thermal expansion or contraction between them. This in turn results in pressurisation and depressurisation of inter vessel gap nitrogen respectively. In order to obtain the external pressurization for the buckling design of MV, transient thermal models for obtaining the evolutions of MV, SV and inter gap nitrogen temperatures and hence their relative thermal expansion and inter vessel gap pressure have been developed. This paper gives the details of the mathematical model, assumptions made in the calculation and the results of the analysis.

  19. Maury Journals - German Vessels

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — German vessels observations, after the 1853 Brussels Conference that set International Maritime Standards, modeled after Maury Marine Standard Observations.

  20. Association of Lupus Anticoagulant With Long‐Term Damage Accrual in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

    National Research Council Canada - National Science Library

    Jordan, Natasha; D'cruz, David P

    2016-01-01

    ... and other immunosuppressive therapies. Box Significance & Innovations Antiphospholipid antibodies, particularly lupus anticoagulant, are prevalent in antineutrophil cytoplasmic antibody–associated vasculitis (AAV) patients. The vasculitis damage index is significantly higher in AAV with persistently positive antiphospholipid antibodies or a concurrent diagnosis of t...

  1. Levamisole-induced occlusive necrotising vasculitis in cocaine abusers: An unusual cause of skin necrosis and neutropenia

    Science.gov (United States)

    Belfonte, Cassius Diego; Shanmugam, Victoria Kate; Kieffer, Nicole; Coker, Shodeinde; Boucree, Suelyn; Kerr, Gail

    2013-01-01

    We present three cases describing the various skin manifestations of presumed levamisole-contaminated cocaine use. Antibody-mediated vasculitis and neutropenia were consistent findings in these cases and repeat exposure resulted in distinct dermatologic complications. This phenomenon of levamisole-induced vasculitis and neutropenia is being increasingly described and has characteristic wound manifestations that must be recognised and treated early. PMID:22716045

  2. 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.

  3. Delayed diagnosis of ocular syphilis that manifested as retinal vasculitis and acute posterior multifocal placoid epitheliopathy

    Directory of Open Access Journals (Sweden)

    Jong Hoon Park

    2013-01-01

    Full Text Available A 55-year-old female presented with bilateral progressive retinal vasculitis. She was on systemic and intravitreal steroids on the basis of uveitis work-up result (negative result including rapid plasma reagin, but her visual acuity continued to deteriorate to light perception only. Ocular examination showed retinal vasculitis, multiple yellow placoid lesions and severe macula edema in both eyes. Repeated work-up revealed positivity of fluorescent treponemal antibody-absorption in serum and subsequently in cerebrospinal fluid. Ocular syphilis was diagnosed. And intravenous penicillin G resulted in rapid resolution of vasculitis and macular edema. To avoid delay in the diagnosis of ocular syphilis, high index of suspicion and repeating serological tests (including both treponemal and non-treponemal tests are warranted.

  4. 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.

  5. Higher Serum Angiotensinogen Is an Indicator of IgA Vasculitis with Nephritis Revealed by Comparative Proteomes Analysis.

    Directory of Open Access Journals (Sweden)

    Xuelian He

    Full Text Available IgA vasculitis (IgAV, previously named as Henoch-Schönlein purpura, is the most common systematic vasculitis with unknown etiology. Lack of appropriate study system and/or animal model limits the understanding of its molecular pathogenesis and hinders the identification of targets for rational therapy, especially for its long-term complication, IgAV nephritis (IgAVN. In this study, we applied comparative analysis of serum proteomes to obtain an insight about disease pathogenesis. This study has utilized high sensitivity nanoscale ultra performance liquid chromatography-mass spectrometry (nanoLC-MS/MS to investigate the alterations in serum proteomic profiles in patients with IgAV (n=6, IgAVN (n=6 and healthy subjects (n=7. The differentially expressed proteins were subjected to functional pathway analysis by PANTHER and DAVID software. We identified 107 differentially expressed proteins among three different groups, and functional analysis suggested that, in addition to earlier reported pathways, such as acute phase response, immune response, complement and blood coagulation pathways, hemostasis and Wnt signaling pathway were probably involved in pathogenesis of IgAV. A few differentially abundant proteins identified, such as C4a, serum amyloid A, angiotensinogen, and kininogen 1, were further validated by ELISA. More importantly, we found that angiotensinogen concentration is correlated with IgAVN and could be used as a potential marker for the progression of IgAV. This is the first report of analyzing the proteomic alterations in IgAV patients and the differentially proteins identified in this study may enhance understanding of the pathology of IgAV and a few of them may be used to monitor disease progression.

  6. Macrophage migration inhibitory factor (MIF) and thyroid hormone alterations in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

    Science.gov (United States)

    Wendt, Mårten; Börjesson, Ola; Avik, Aune; Bratt, Johan; Anderstam, Björn; Qureshi, Abdul R; Miller, Edmund J; Gunnarsson, Iva; Bruchfeld, Annette

    2013-05-20

    Macrophage migration inhibitory factor (MIF) is a proinflammatory cytokine known to be released from lymphocytes, macrophages and endothelial cells and also in animal models shown to be inducible with glucocorticoids (GC). In contrast, thyroxine seems to antagonize MIF activity. To investigate whether MIF is increased in active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and possible correlations with GC dosing and thyroid hormone levels, 27 consecutive patients with active AAV were studied and followed prospectively. Disease activity was assessed using Birmingham Vasculitis Activity Score 2003 (BVAS) at baseline and at follow-up at 3 and 6 months, along with MIF, thyroid hormones free triiodothyronine (fT3) and free thyroxine (fT4), C-reactive protein (CRP) and creatinine. MIF was elevated significantly at baseline compared with follow-up at 3 and 6 months (8,618 pg/mL versus 5,696 and 6,212 respectively; P < 0.002) but did not correlate to CRP, GC dose, creatinine or organ involvement. fT3 was depressed significantly at baseline compared with follow-up (1.99 pg/mL versus 2.31 and 2.67 respectively; P = 0.01) and correlated inversely to the BVAS score at baseline. We found a significant correlation between the MIF/fT4 ratio at baseline versus MIF/fT4 ratio at 6 months (ρ = 0.52, P < 0.005) and a trend between the baseline MIF/fT3 ratio versus MIF/fT3 ratio at 6 months (ρ = 0.39, P = 0.05). These results suggest a possible role for MIF and thyroid status in AAV. Further studies could reveal whether the association between AAV and thyroid hormone levels in the context of elevated MIF may present a link as well as a target of treatment.

  7. Febrile ulceronecrotic Mucha-Habermann disease with central nervous system vasculitis.

    Science.gov (United States)

    Rosman, Ilana S; Liang, Ling-Chih; Patil, Sushama; Bayliss, Susan J; White, Andrew J

    2013-01-01

    Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). We report a case of FUMHD in an 11-year-old boy who subsequently developed neurologic symptoms and was found to have necrotizing vasculitis on brain biopsy. Over 5 years of follow-up, he had one biopsy-proven recurrence of his skin lesions and continued rehabilitation and treatment for residual neurologic complications. This case provides histological evidence of central nervous system vasculitis associated with FUMHD. Our patient's disease was eventually controlled with cyclophosphamide. © 2012 Wiley Periodicals, Inc.

  8. Connective tissue disorders associated with vasculitis and vaso-occlusive disease of the hand.

    Science.gov (United States)

    Michelotti, Brett; Rizzo, Marco; Moran, Steven L

    2015-02-01

    Hand ischemia caused by vasculitis is a secondary finding in many autoimmune processes. Many of these autoimmune diseases are managed primarily with medications that can prevent the development of occlusive disease, tissue ischemia, and tissue loss. Unfortunately several disease conditions can be recalcitrant to medical management and can result in ischemic changes within the hand, which may require operative intervention. This article briefly reviews the major connective tissue disorders associated with vasculitis and vaso-occlusive disease of the hand, including scleroderma, lupus, and Buerger disease, and their surgical treatment. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. 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.

  10. A case of hypocomplementaemic urticarial vasculitis with a high serum level of rheumatoid factor.

    Science.gov (United States)

    Ashida, Atsuko; Murata, Hiroshi; Ohashi, Atsuko; Ogawa, Eisaku; Uhara, Hisashi; Okuyama, Ryuhei

    2013-08-01

    We report a case of hypocomplementaemic urticarial vasculitis with an elevated serum rheumatoid factor level. Hypocomplementaemic urticarial vasculitis is an immune complex-mediated disease characterised by urticarial eruptions. High levels of rheumatoid factor may be associated with hypocomplementaemia due to the consumption of complement, because the rheumatoid factor can form immune complexes with immunoglobulin. It is necessary to pay attention to the amounts of complement in cases of urticarial eruptions with elevated rheumatoid factor level. The eruptions were relieved with a combination of prednisolone and colchicine. © 2012 The Authors. Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists.

  11. Unusual exanthema combined with cerebral vasculitis in pneumococcal meningitis: a case report

    Directory of Open Access Journals (Sweden)

    Raissaki Maria

    2011-08-01

    Full Text Available Abstract Introduction Bacterial meningitis is a complex, rapidly progressive disease in which neurological injury is caused in part by the causative organism and in part by the host's own inflammatory responses. Case presentation We present the case of a two-year-old Greek girl with pneumococcal meningitis and an atypical curvilinear-like skin eruption, chronologically associated with cerebral vasculitis. A diffusion-weighted MRI scan showed lesions with restricted diffusion, reflecting local areas of immunologically mediated necrotizing vasculitis. Conclusions Atypical presentations of bacterial meningitis may occur, and they can be accompanied by serious unexpected complications.

  12. PRESSURE-RESISTANT VESSEL

    NARCIS (Netherlands)

    Beukers, A.; De Jong, T.

    1997-01-01

    Abstract of WO 9717570 (A1) The invention is directed to a wheel-shaped pressure-resistant vessel for gaseous, liquid or liquefied material having a substantially rigid shape, said vessel comprising a substantially continuous shell of a fiber-reinforced resin having a central opening, an inner

  13. Containment vessel drain system

    Energy Technology Data Exchange (ETDEWEB)

    Harris, Scott G.

    2018-01-30

    A system for draining a containment vessel may include a drain inlet located in a lower portion of the containment vessel. The containment vessel may be at least partially filled with a liquid, and the drain inlet may be located below a surface of the liquid. The system may further comprise an inlet located in an upper portion of the containment vessel. The inlet may be configured to insert pressurized gas into the containment vessel to form a pressurized region above the surface of the liquid, and the pressurized region may operate to apply a surface pressure that forces the liquid into the drain inlet. Additionally, a fluid separation device may be operatively connected to the drain inlet. The fluid separation device may be configured to separate the liquid from the pressurized gas that enters the drain inlet after the surface of the liquid falls below the drain inlet.

  14. Vasculitis, cerebral infarction and persistent Bartonella henselae infection in a child.

    Science.gov (United States)

    Balakrishnan, Nandhakumar; Ericson, Marna; Maggi, Ricardo; Breitschwerdt, Edward B

    2016-05-10

    The genus Bartonella is comprised of a rapidly increasing number of pathogenic species that induce a seemingly diverse spectrum of neurological symptoms. During the 12 year period that followed the initial onset of neurological and gastrointestinal symptoms, an 11 year-old girl experienced a spectrum of neurological complaints including frequent headaches, visual and auditory hallucinations, anxiety, vision loss involving the lower left quadrant of both eyes, episodic bouts of generalized paralysis, facial palsy, chronic insomnia, seizures, dizziness, cognitive dysfunction, and memory loss. PCR assays targeting Bartonella spp. were used to test formalin-fixed, paraffin embedded brain tissue, patient blood specimens and Bartonella alpha Proteobacteria growth medium (BAPGM) enrichment blood cultures. PCR positive amplicons were sequenced directly and compared to GenBank sequences. Bartonella spp. serology was performed by indirect fluorescent antibody testing and confocal laser scanning microscopy was used to visualize B. henselae organisms in resected brain. Bartonella henselae DNA was independently PCR amplified and sequenced from the girl's right parietal lobe, surgically resected in 2000 and from a blood specimen collected in 2012. Although causation cannot be established by a case report, prior diagnostic testing resulted in findings that were either inconclusive or within normal reference ranges and no etiological diagnosis had been obtained to explain the patient's initial or progressive neurological symptoms. As intravascular, intra-erythrocytic and endotheliotropic bacteria, it is possible that B. henselae initially induced a vasculitis, resulting in secondary cerebral infarction, tissue necrosis and surgical resection. Bartonella bacteremia, potentially spanning a 12-year time frame, in conjunction with the therapeutic administration of immunosuppressive drugs may have resulted in a progression and potentiation of the neurological disease that was

  15. Digital vasculitis in systemic lupus erythematosus: a minor manifestation of disease activity?

    Science.gov (United States)

    Gomes, C; Carvalho, J F; Borba, E F; Borges, C T L; Vendramini, M B; Bueno, C; Costa, L P; Bonfá, E

    2009-10-01

    The objective of this study is to determine if digital vasculitis (DV), a clinical manifestation with a high systemic lupus erythematosus disease activity index (SLEDAI) score, is associated with lupus severity. DV and other clinical manifestations defined according to the SLEDAI were evaluated in 168 consecutive patients with systemic lupus erythematosus (SLE). Two groups were defined according to presence (DV+, n = 27) or absence of DV (DV-, n = 141) at the time of evaluation. The exclusion criterion was the presence of antiphospholipid syndrome (Sapporo's criteria). The two groups were comparable with regard to age (P = 0.09), gender (P = 1.00), white race (P = 0.81), and disease duration (P = 0.78). Compared to the DV- group, the DV+ group had a significantly higher frequency of mucocutaneous manifestations (66.7 vs. 39.0%, P = 0.01), haematological abnormalities (22.2 vs. 6.4%, P = 0.02) and constitutional symptoms (11.1 vs. 0.7%, P = 0.01). Renal and neurological involvements were similar in both groups (P = 0.57 and P = 1.00, respectively). The evaluation of each SLEDAI parameter confirmed that the DV+ group had higher frequencies of mild manifestations, such as new rash (P = 0.02), alopecia (P = 0.02), oral ulcers (P = 0.045), fever (P = 0.01) and leucopenia (P = 0.005). In contrast, both groups had similarly increased anti-dsDNA (P = 0.78) and decreased complement levels (P = 0.29). In conclusion, DV in patients with SLE identifies a subgroup of a mild disease. The high 'weighted' index attributed to this alteration in the SLEDAI score should therefore be revised.

  16. Recent Advancements in Retinal Vessel Segmentation.

    Science.gov (United States)

    L Srinidhi, Chetan; Aparna, P; Rajan, Jeny

    2017-04-01

    Retinal vessel segmentation is a key step towards the accurate visualization, diagnosis, early treatment and surgery planning of ocular diseases. For the last two decades, a tremendous amount of research has been dedicated in developing automated methods for segmentation of blood vessels from retinal fundus images. Despite the fact, segmentation of retinal vessels still remains a challenging task due to the presence of abnormalities, varying size and shape of the vessels, non-uniform illumination and anatomical variability between subjects. In this paper, we carry out a systematic review of the most recent advancements in retinal vessel segmentation methods published in last five years. The objectives of this study are as follows: first, we discuss the most crucial preprocessing steps that are involved in accurate segmentation of vessels. Second, we review most recent state-of-the-art retinal vessel segmentation techniques which are classified into different categories based on their main principle. Third, we quantitatively analyse these methods in terms of its sensitivity, specificity, accuracy, area under the curve and discuss newly introduced performance metrics in current literature. Fourth, we discuss the advantages and limitations of the existing segmentation techniques. Finally, we provide an insight into active problems and possible future directions towards building successful computer-aided diagnostic system.

  17. Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy

    Science.gov (United States)

    Murray, Nigel P.; Ruíz, Amparo; Reyes, Eduardo

    2012-01-01

    Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor. PMID:22919534

  18. Hypernephroma Presenting with Cutaneous Leukocytoclastic Vasculitis and Lupus Anticoagulant: Resolution after Nephrectomy

    Directory of Open Access Journals (Sweden)

    Nigel P. Murray

    2012-01-01

    Full Text Available Hypernephroma can present as a variety of paraneoplastic, nonmetastatic conditions, including vasculitis, and rarely a lupus-type anticoagulant. Nephrectomy leads to the resolution of the systemic complaints. Malignancy, in this case hypernephroma, can present as an immune-mediated paraneoplastic syndrome which resolves after removal of the underlying tumor.

  19. 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...

  20. Venous thromboembolism in ANCA-associated vasculitis - incidence and risk factors

    NARCIS (Netherlands)

    Stassen, P. M.; Derks, R. P. H.; Kallenberg, C. G. M.; Stegeman, C. A.

    Objectives. In patients with ANCA-associated vasculitis (AAV), an increased incidence of venous thromboembolism (VTE), mainly during active disease, has been described. In a large cohort of AAV patients, live assessed the incidence of VTE and its relation with disease activity and classic risk

  1. The incidence of vasculitis is increased in female stroke-prone ...

    African Journals Online (AJOL)

    Background: Vascular changes in hypertension share common characteristics with inflammatory wall injury. Since it is known that chronic inflammatory diseases are frequently more prevalent in females, this study tested the hypothesis that vasculitis would be more evident in female stroke-prone spontaneously hypertensive ...

  2. Aerococcus viridans infection presenting as cutaneous vasculitis in an immunocompetent patient.

    Science.gov (United States)

    Parrey, Ashaq Hussain; Sofi, Fayaz; Ahmad, Mushtaq; Kuchay, Abid

    2016-01-01

    Aerococcus viridans organisms are Gram-positive cocci that are widely distributed in hospital environments and room air. These bacteria have infrequently been encountered as human pathogens causing bacteremia, endocarditis and urinary tract infections. The significance of these bacteria may be overlooked due to their fastidious growth, and they are often confused with other strains of streptococci or staphylococci. We present a case of Aerococcus viridans manifesting as cutaneous vasculitis in an immunocompetent patient. A 30-year-old female patient was admitted to hospital after two weeks history of fever, chills and papular rash over the limbs and trunk. The clinical diagnosis of vasculitis was made. Investigations revealed elevated leucocytosis (21.7 × 10(9)/l) with 81% of neutrophils, and an elevated erythrocyte sedimentation rate or 60 mm/h. Serum anti-neutrophil cytoplasmic antibodies (ANCAs) were not found. Blood culture showed growth of Aerococcus viridans. Histopathological assessment of skin biopsy revealed cutaneous vasculitis. To date, no clinical case report of this kind has been reported implicating Aerococcus viridans in cutaneous vasculitis. Increased awareness and more studies of this genus should lead to the identification of its potential role in human infections.

  3. Patients with systemic vasculitis have increased levels of autoantibodies against oxidized LDL

    NARCIS (Netherlands)

    Swets, BP; Brouwer, DAJ; Tervaert, JWC

    Oxidation of low density lipoprotein (LDL) is considered to play an important role in the development of atherosclerosis and increased levels of autoantibodies against oxidized LDL have been found in patients with various manifestations of atherosclerosis. Patients with vasculitis are prone to the

  4. Urticaria vasculitis in a child: a case report and literature review.

    Science.gov (United States)

    Imbernón-Moya, Adrián; Vargas-Laguna, Elena; Burgos, Fernando; Fernández-Cogolludo, Eva; Aguilar-Martínez, Antonio; Gallego-Valdés, Miguel Ángel

    2017-08-01

    Annular urticarial lesions in a child must establish a main differential diagnosis with urticaria multiforme, common urticaria, acute hemorrhagic edema of infancy, erythema marginatum, erythema annulare centrifugum, annular erythema in childhood, erythema multiforme, Sweet's syndrome, Schönlein-Henoch purpura, erythematosus lupus, several systemic vasculitis, and serum sickness.

  5. Pulmonary capillaritis and its relationship to development of emphysema in hypocomplementaemic urticarial vasculitis syndrome.

    Science.gov (United States)

    Hunt, David P J; Weil, Rimona; Nicholson, Andrew G; Burke, Margaret M; Du Bois, Ron M; Wells, Athol U

    2006-03-01

    Hypocomplementaemic urticarial vasculitis syndrome (HUVS) is a rare disorder characterised by complement activation and the presence of C1q precipitins together with a syndrome of urticarial vasculitis, angioedema, arthralgia, ocular inflammation, glomerulonephritis and obstructive lung disease. The pathophysiology of the obstructive airways disease is poorly understood. We report a 46 year-old woman with HUVS who developed progressive obstructive airways disease. Lung biopsy early in the course of her disease revealed pulmonary capillaritis. The disease progressed despite treatment with steroids and cyclosporin and the patient eventually underwent successful double lung transplantation. The explanted lung showed the coexistence of a patchy active vasculitis with severe panacinar emphysema. This is the first description of the histopathological process of HUVS in an explanted lung. Through analysis of serial histopathological specimens and clinical data we show the evolution of pulmonary capillaritis to emphysema, and demonstrate that active vasculitis can coexist with emphysema in patients with HUVS and obstructive airways disease. We suggest that there is a role for ongoing immunosuppressive therapy in these patients.

  6. Rapid Growth of Lung Nodules due to Combined Pulmonary Vasculitis, Silicoanthracosis, and Chondrocalcinosis

    Directory of Open Access Journals (Sweden)

    Wolfgang Jungraithmayr

    2016-01-01

    Full Text Available Background. Silicoanthracosis is a pneumoconiosis due to occupational inhalation of silica and carbon dusts. Clinically, it can be associated with vasculitis or rheumatoid arthritis. In association with these diseases, silicoanthracosis can present within the lung with multiple pulmonary nodules which, as a differential diagnosis, can mimic metastatic disease or multiple abscesses. Case Presentation. We present the case of a 62-year old former pit worker with pulmonary nodules, chondrocalcinosis due to calcium pyrophosphate deposition (CPPD, and a history of renal cancer. Within a short period of time, pulmonary nodules grew rapidly. Thoracoscopically, the resected lung specimen revealed silicoanthracosis associated with small-to-medium-size vasculitis in the presence of antineutrophil cytoplasmatic autoantibodies (c-ANCA. Conclusion. Pulmonary silicoanthracotic lesions on the base of ANCA-associated vasculitis and CPPD arthritis can rapidly grow. A mutual correlation between silicoanthracosis, ANCA-associated vasculitis, and CPPD seems possible. Apart from this, consideration of metastatic disease should be obligatory in patients with a history of cancer at the same time being immunosuppressed.

  7. 2013 Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  8. 2011 Passenger Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  9. 2011 Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  10. 2013 Passenger Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  11. 2013 Tanker Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  12. 2013 Cargo Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  13. High Performance Marine Vessels

    CERN Document Server

    Yun, Liang

    2012-01-01

    High Performance Marine Vessels (HPMVs) range from the Fast Ferries to the latest high speed Navy Craft, including competition power boats and hydroplanes, hydrofoils, hovercraft, catamarans and other multi-hull craft. High Performance Marine Vessels covers the main concepts of HPMVs and discusses historical background, design features, services that have been successful and not so successful, and some sample data of the range of HPMVs to date. Included is a comparison of all HPMVs craft and the differences between them and descriptions of performance (hydrodynamics and aerodynamics). Readers will find a comprehensive overview of the design, development and building of HPMVs. In summary, this book: Focuses on technology at the aero-marine interface Covers the full range of high performance marine vessel concepts Explains the historical development of various HPMVs Discusses ferries, racing and pleasure craft, as well as utility and military missions High Performance Marine Vessels is an ideal book for student...

  14. Cheboygan Vessel Base

    Data.gov (United States)

    Federal Laboratory Consortium — Cheboygan Vessel Base (CVB), located in Cheboygan, Michigan, is a field station of the USGS Great Lakes Science Center (GLSC). CVB was established by congressional...

  15. Maury Journals - US Vessels

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — U.S. vessels observations, after the 1853 Brussels Conference that set International Maritime Standards, modeled after Maury Marine Standard Observations.

  16. 2011 Cargo Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  17. 2011 Tanker Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  18. 2013 Fishing Vessel Density

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Automatic Identification Systems (AIS) are a navigation safety device that transmits and monitors the location and characteristics of many vessels in U.S. and...

  19. Coastal Logbook Survey (Vessels)

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — This data set contains catch (landed catch) and effort for fishing trips made by vessels that have been issued a Federal permit for the Gulf of Mexico reef fish,...

  20. LANL Robotic Vessel Scanning

    Energy Technology Data Exchange (ETDEWEB)

    Webber, Nels W. [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2015-11-25

    Los Alamos National Laboratory in J-1 DARHT Operations Group uses 6ft spherical vessels to contain hazardous materials produced in a hydrodynamic experiment. These contaminated vessels must be analyzed by means of a worker entering the vessel to locate, measure, and document every penetration mark on the vessel. If the worker can be replaced by a highly automated robotic system with a high precision scanner, it will eliminate the risks to the worker and provide management with an accurate 3D model of the vessel presenting the existing damage with the flexibility to manipulate the model for better and more in-depth assessment.The project was successful in meeting the primary goal of installing an automated system which scanned a 6ft vessel with an elapsed time of 45 minutes. This robotic system reduces the total time for the original scope of work by 75 minutes and results in excellent data accumulation and transmission to the 3D model imaging program.

  1. Cardiac magnetic resonance imaging showing complete resolution of subendocardial involvement in Churg-Strauss syndrome.

    Science.gov (United States)

    Post, Martijn C; Boomsma, Martijn F; van Heesewijk, Johannes P M; Grutters, Jan C; Van der Heyden, Jan

    2011-08-01

    Churg-Strauss syndrome or allergic angiitis and granulomatosis is a rare systemic vasculitis. Cardiac involvement is the leading cause of mortality and includes eosinophilic endomyocarditis. We present a case of complete resolution of subendocardial involvement after high-dose corticosteroids, diagnosed by contrast-enhanced cardiovascular magnetic resonance imaging.

  2. Risk Assessment for Fishing Vessels at Fishing Grounds

    Directory of Open Access Journals (Sweden)

    Sergey Moyseenko

    2015-09-01

    Full Text Available Safety and efficiency of fishing fleet activity depend largely on the quality of management decisions. Cause-and-effect relationships of accidents involving fishing vessels were identified by means of an analysis of emergencies and fishing incidents. The suggested method of risks calculation is based on the use of statistical methods, fuzzy sets/expert estimations method and the probability theory. The following most common tasks are presented and solved: - there is an impact of two or more independent negative factors/events on the vessel such as failure of a sonar, a vessel operator error, another vessel operator error. - a transport vessel carries out loading and unloading of fishing vessels under different environmental conditions. The value of the risk of an emergency incident is determined. - the fishing vessel navigation performs under various meteorological conditions. A priori probability of incident-free operation is calculated according to expert estimations.

  3. Randomized trial of plasma exchange or high-dosage Methylprednisolone as adjunctive therapy for severe renal vasculitis

    NARCIS (Netherlands)

    Jayne, David R. W.; Gaskin, Gill; Rasmussen, Niels; Abramowicz, Daniel; Ferrario, Franco; Guillevin, Loic; Mirapeix, Eduardo; Savage, Caroline O. S.; Sinico, Renato A.; Stegeman, Coen A.; Westman, Kerstin W.; van der Woude, Fokko J.; van Wijngaarden, Robert A. F. de Lind; Pusey, Charles D.

    Systemic vasculitis associated with autoantibodies to neutrophil cytoplasmic antigens (ANCA) is the most frequent cause of rapidly progressive glomerulonephritis. Renal failure at presentation carries an increased risk for ESRD and death despite immunosuppressive therapy. This study investigated

  4. A case of central nervous system vasculitis related to an episode of Guillain-Barrè syndrome

    OpenAIRE

    Sinardi, Daniele; Spada, Antonella; Marino, Antonella; Mondello, Epifanio

    2000-01-01

    The authors report their knowledge about an uncommon case of isolated vasculitis, restricted to the left sylvian artery during an auto-immune Guillain-Barrè syndrome (GBS), sustained by cytomegalovirus (CMV). An acute cardiopulmonary failure requiring a ventilator and vasopressor support manifested, notwithstanding plasma exchanging and immune-modulating therapy. An IgM-enriched formula administration coincided with a rapid amelioration of GBS and vasculitis to a complete recovery the next mo...

  5. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature

    Science.gov (United States)

    Al Mosawi, Zakiya Saleh Adnan; Al Hermi, Badriya Ebrahim Ahmed

    2013-01-01

    Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate. PMID:23904922

  6. Hypocomplementemic Urticarial Vasculitis Syndrome in an 8-year-old Boy: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Zakiya Saleh Adnan Al Mosawi

    2013-07-01

    Full Text Available Hypocomplementemic urticarial vasculitis syndrome is an immune complex-mediated disease of unknown etiology. The clinical course is characterized by urticaria, conjunctivitis, joint pain, and hypocomplementemia. We here report a case of a child with hypocomplementemic urticarial vasculitis syndrome that progressed to nephritis. Renal biopsy was consistent with diffuse proliferative glomerulonephritis with diffuse subendothelial immune deposits. He responded well to a combination of steroid and mofetil micofenolate.

  7. 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

    Full Text Available Introduction: Few well-documented cases of central nervous system involvement in patients with mixed cryoglobulinemia and/or HCV infection have been reported. We can distinguish between acute or subacute diffuse and focal lesions (transient ischemic attack-like syndromes and cerebrovascular accidents. Methods: A search of two electronic databases (Medline and EMBASE was conducted from the year of their inception (1966 for Medline and 1988 for EMBASE to September 2000. The search strategy employed entailed combining these terms: Cryoglobulinemia, Central Nervous System, Hepatitis C, chronic hepatitis. Cryoglobulinemia and Central Nervous System were also used as free test words. We analysed articles with case reports and the most frequent articles on the references list. Pathogenesis: The main pathophysiologic mechanism of cerebral involvement is ischemia (or rarely hemorrhage due to diffuse or segmental vasculitis of the small cerebral vessels. In these cases a brain MRI usually shows single or multiple increased T2 signals. Furthermore an occasional occlusive vasculopathy without vasculitis was documented histologically. In these patients ischemia could be started or enhanced by the engorgement of the microvasculature by clumps of red cells and by aggregates of cryoglobulins. In the same patients vasculitis and hemoreological abnormalities can affect the clinical picture of the cerebral involvement in mixed cryoglobulinemia. Finally, the detection of HCV in the lesions induces a hypothesis that, in some cases, CNS involvement could be directly related to chronic HCV infection, even in the absence of cryoglobulin production. Case report: We describe a 63 year-old woman with acute severe encephalopathy. Laboratory evaluation revealed a high positive test result for rheumatoid factor (3390 U/ml and hypocomplementemia (C4 less than 1.67 mg/dl. Protein immunofixation electrophoresis demonstrated 5% monoclonal proteins (IgM/k and IgG/k, 3

  8. Integrating Multiple Autonomous Underwater Vessels, Surface Vessels and Aircraft into Oceanographic Research Vessel Operations

    Science.gov (United States)

    McGillivary, P. A.; Borges de Sousa, J.; Martins, R.; Rajan, K.

    2012-12-01

    Autonomous platforms are increasingly used as components of Integrated Ocean Observing Systems and oceanographic research cruises. Systems deployed can include gliders or propeller-driven autonomous underwater vessels (AUVs), autonomous surface vessels (ASVs), and unmanned aircraft systems (UAS). Prior field campaigns have demonstrated successful communication, sensor data fusion and visualization for studies using gliders and AUVs. However, additional requirements exist for incorporating ASVs and UASs into ship operations. For these systems to be optimally integrated into research vessel data management and operational planning systems involves addressing three key issues: real-time field data availability, platform coordination, and data archiving for later analysis. A fleet of AUVs, ASVs and UAS deployed from a research vessel is best operated as a system integrated with the ship, provided communications among them can be sustained. For this purpose, Disruptive Tolerant Networking (DTN) software protocols for operation in communication-challenged environments help ensure reliable high-bandwidth communications. Additionally, system components need to have considerable onboard autonomy, namely adaptive sampling capabilities using their own onboard sensor data stream analysis. We discuss Oceanographic Decision Support System (ODSS) software currently used for situational awareness and planning onshore, and in the near future event detection and response will be coordinated among multiple vehicles. Results from recent field studies from oceanographic research vessels using AUVs, ASVs and UAS, including the Rapid Environmental Picture (REP-12) cruise, are presented describing methods and results for use of multi-vehicle communication and deliberative control networks, adaptive sampling with single and multiple platforms, issues relating to data management and archiving, and finally challenges that remain in addressing these technological issues. Significantly, the

  9. A case of urticarial vasculitis in a female patient with lupus: Mycoplasma pneumoniae infection or lupus reactivation?

    Science.gov (United States)

    Diplomatico, Mario; Gicchino, Maria Francesca; Ametrano, Orsola; Marzuillo, Pierluigi; Olivieri, Alma Nunzia

    2017-05-01

    A 17-year-old female patient affected by systemic lupus erythematosus (SLE) (who had been taking 300 mg/die of hydroxychloroquine for 3 years), Graves' disease (treated with 10 mg/die of tapazole), and celiac disease came to our attention for urticarial vasculitis. She had been taking prednisone (25 mg/die) for 3 days, and her blood tests showed high levels of Mycoplasma pneumoniae IgM and IgG antibodies. The association between urticaria and M. pneumoniae infections can be present in up to 7% of the cases and, to the best of our knowledge, only two reports of urticarial vasculitis and M. pneumoniae in adults are available in the literature. Urticarial vasculitis can also be a rare cutaneous manifestation of SLE (affecting 2% of the patients), and our case is the first in the literature describing the coexistence of M. pneumoniae infection, SLE, and urticarial vasculitis in a pediatric patient, a case that rises an important differential diagnosis issue about the origin of urticarial vasculitis: SLE reactivation or urticarial vasculitis due to M. pneumoniae infection?

  10. 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.

  11. Ultraviolet erythema as an indicator of nonspecific reactivity during hemorrhagic vasculitis in children

    Energy Technology Data Exchange (ETDEWEB)

    Kharitonova, A.V.

    1973-01-01

    From 1955 to 1967 studies were carried out on 407 children suffering from hemorrhagic vasculitis. For patients in the severe stage of hemorrhagic vasculitis it is characteristic that there are functional shifts in the central nervous system, which are manifested by a changed sensitivity of the skin to ultraviolet irradiation. The appearance of slowed, inert or paradoxal reactions indicates the predominance of processes in the brain that slow down and decrease the reactivity of the organism. In rheumatoid and abdominal-rheumatoid syndromes of the disease, disturbances in indices to skin light-sensitivity are the most manifest. During the recovery period, the indices of ultraviolet erythema, on approaching normal values, still remain low. This behavior requires extensive outpatient observation and treatment of sick children to avoid recidivism. 9 references. (SJR)

  12. Levamisole-induced myopathy and leukocytoclastic vasculitis: a case report and literature review.

    Science.gov (United States)

    Tsai, Meng-Hsuan; Yang, Jen-Hung; Kung, Sheng-Ling; Hsiao, Yu-Ping

    2013-01-01

    Levamisole, an immunomodulator and anthelmintic medication, has been used in dermatology for years. Even though the adverse effects are usually mild and reversible, attention should be paid toward severe events such as vasculitis and neutropenia. To the best of our knowledge, this is the first case report on a patient presenting with myopathy caused by levamisole. Here, we report a 34-year-old woman with recalcitrant warts who received levamisole 100 mg daily for 5 days. Subsequently, bilateral lower limb weakness accompanied by multiple painful and non-blanchable purpura was noted. Levamisole-induced myopathy and leukocytoclastic vasculitis were diagnosed by skin histopathology, direct immunofluorescence, and electromyography. After discontinuing levamisole and giving a short course of systemic steroid, these symptoms demonstrated a resolving trend. © 2013 Wiley Periodicals, Inc.

  13. Membranous nephropathy with crescents associated with levamisole-induced MPO-ANCA vasculitis.

    Science.gov (United States)

    Moinuddin, Irfan; Madhrira, Machaiah; Bracamonte, Erika; Thajudeen, Bijin; Sussman, Amy

    2016-07-01

    ANCA-associated vasculitis (AAV) is the most common cause of crescentic rapidly progressive glomerulonephritis (GN). Levamisole used as an adulterant in cocaine is increasingly recognized as a cause of AAV. We report the case of a 50 year old woman with atypical anti-MPO AAV associated with cocaine use and exposure to levamisole. In addition to the clinical and pathologic findings of crescentic GN, the patient also had biopsy evidence of secondary membranous nephropathy (MN). Although AAV and MN have been reported previously in the same patient and both have been induced by drug exposures, this is the first report of MN in a patient with AAV likely induced by levamisole. We suggest that MPO can cause both pauci-immune vasculitis and secondary membranous nephropathy in some cases, as in cases of levamisole-adulterated cocaine use. Copyright © 2016 Elsevier GmbH. All rights reserved.

  14. Mycoplasma pneumoniae infection associated with urticarial vasculitis mimicking adult-onset Still's disease.

    Science.gov (United States)

    Dua, Janet; Nandagudi, Anupama; Sutcliffe, Nurhan

    2012-12-01

    Mycoplasma pneumoniae is well known to be a frequent cause of atypical pneumonia worldwide. However, it may also present with a wide variety of clinical features, including cutaneous symptoms, which are not widely recognised. Urticarial vasculitis occurring with M. pneumoniae has been described to occur in only one other case report. This amalgamation of non-specific clinical symptoms and signs can lead to a diagnostic dilemma. We describe a case of M. pneumoniae infection presenting with extrapulmonary manifestations and urticarial vasculitis, which was misdiagnosed as adult-onset Still's disease (AOSD). Had immunosuppressive therapy been commenced for AOSD in the presence of undiagnosed infection, this may have resulted in potentially serious consequences. This case highlights the need to remain vigilant about diagnosing M. pneumoniae as its serological diagnosis may take weeks and it has many extrapulmonary manifestations, which can masquerade as other conditions.

  15. Isolated periostitis as a manifestation of systemic vasculitis in a child: imaging features.

    Science.gov (United States)

    Cheon, Jung-Eun; Kim, In-One; Kim, Woo Sun; Yeon, Kyung Mo

    2010-12-01

    We report a case of isolated periostitis associated with Takayasu arteritis in a 10-year-old boy presenting with calf pain. Radiographs revealed a localized, thick periosteal reaction with irregular margin in the proximal left fibula. MRI revealed irregular thickening with enhancement of the periosteum in the proximal fibula and heterogeneous enhancement along vascular bundles in the calf. Isolated periostitis is a rare skeletal manifestation of systemic vasculitis that could be misdiagnosed as neoplastic or traumatic periosteal reaction.

  16. Isolated periostitis as a manifestation of systemic vasculitis in a child: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Jung-Eun; Kim, In-One; Kim, Woo Sun; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, 101 Daehang-ro Jongro-gu, Seoul (Korea, Republic of)

    2010-12-15

    We report a case of isolated periostitis associated with Takayasu arteritis in a 10-year-old boy presenting with calf pain. Radiographs revealed a localized, thick periosteal reaction with irregular margin in the proximal left fibula. MRI revealed irregular thickening with enhancement of the periosteum in the proximal fibula and heterogeneous enhancement along vascular bundles in the calf. Isolated periostitis is a rare skeletal manifestation of systemic vasculitis that could be misdiagnosed as neoplastic or traumatic periosteal reaction. (orig.)

  17. 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...... in severe nephritis, when used in conjunction with immunosuppressive drug therapy. The development of PLEX for this indication, the strength of the clinical trial evidence supporting its use, its roles in other AAV indications and ongoing research are discussed....

  18. Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants

    Directory of Open Access Journals (Sweden)

    Stathopoulos Konstantinos

    2011-09-01

    Full Text Available Abstract Background Secondary systemic vasculitis after posterior spinal fusion surgery is rare. It is usually related to over-reaction of immune-system, to genetic factors, toxicity, infection or metal allergies. Case Description A 14 year-old girl with a history of extended posterior spinal fusion due to idiopathic scoliosis presented to our department with diffuse erythema and nephritis (macroscopic hemuresis and proteinuria 5 months post surgery. The surgical trauma had no signs of inflammation or infection. The blood markers ESR and CRP were increased. Skin tests were positive for nickel allergy, which is a content of titanium alloy. The patient received corticosteroids systematically (hydrocortisone 10 mg for 6 months, leading to total recess of skin and systemic reaction. However, a palpable mass close to the surgical wound raised the suspicion of a late infection. The patient had a second surgery consisting of surgical debridement and one stage revision of posterior spinal instrumentation. Intraoperative cultures were positive to Staphylococcus aureus. Intravenous antibiotics were administered. The patient is now free of symptoms 24 months post revision surgery without any signs of recurrence of either vasculitis or infection. Literature Review Systemic vasculitis after spinal surgery is exceptionally rare. Causative factors are broad and sometimes controversial. In general, it is associated with allergy to metal ions. This is usually addressed with metal on metal total hip bearings. In spinal surgery, titanium implants are considered to be inert and only few reports have presented cases with systemic vasculitides. Therefore, other etiologies of immune over-reaction should always be considered, such as drug toxicity, infection, or genetic predisposition. Purposes and Clinical Relevance Our purpose was to highlight the difficulties during the diagnostic work-up for systemic vasculitis and management in cases of posterior spinal surgery.

  19. Disseminated Burkholderia gladioli infection in a lung transplant recipient with underlying hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Thompson, G R; Wickes, B L; Herrera, M L; Haman, T C; Lewis, J S; Jorgensen, J H

    2011-12-01

    Burkholderia gladioli is difficult to definitively identify within the laboratory using phenotypic testing alone. We describe a case of recurrent B. gladioli infection in a lung transplant recipient with underlying hypocomplementemic urticarial vasculitis syndrome, discuss the difficulties encountered with laboratory identification, provide a review of the methodology required for definitive identification, and discuss potential pathophysiologic mechanisms in this patient responsible for the difficulty in treatment. © 2011 John Wiley & Sons A/S.

  20. Unusual exanthema combined with cerebral vasculitis in pneumococcal meningitis: a case report

    OpenAIRE

    Raissaki Maria; Geromarkaki Elisabeth; Ilia Stavroula; Spanaki Anna-Maria; Tavladaki Theonimfi; Briassoulis George

    2011-01-01

    Abstract Introduction Bacterial meningitis is a complex, rapidly progressive disease in which neurological injury is caused in part by the causative organism and in part by the host's own inflammatory responses. Case presentation We present the case of a two-year-old Greek girl with pneumococcal meningitis and an atypical curvilinear-like skin eruption, chronologically associated with cerebral vasculitis. A diffusion-weighted MRI scan showed lesions with restricted diffusion, reflecting local...

  1. GOLD PRESSURE VESSEL SEAL

    Science.gov (United States)

    Smith, A.E.

    1963-11-26

    An improved seal between the piston and die member of a piston-cylinder type pressure vessel is presented. A layer of gold, of sufficient thickness to provide an interference fit between the piston and die member, is plated on the contacting surface of at least one of the members. (AEC)

  2. Network of endocardial vessels.

    Science.gov (United States)

    Lee, Byung-Cheon; Kim, Hong Bae; Sung, Baeckkyoung; Kim, Ki Woo; Sohn, Jamin; Son, Boram; Chang, Byung-Joon; Soh, Kwang-Sup

    2011-01-01

    Although there have been reports on threadlike structures inside the heart, they have received little attention. We aimed to develop a method for observing such structures and to reveal their ultrastructures. An in situ staining method, which uses a series of procedures of 0.2-0.4% trypan blue spraying and washing, was applied to observe threadlike structures on the surfaces of endocardia. The threadlike structures were isolated and observed by using confocal laser scanning microscopy (CLSM) and transmission electron microscopy (TEM). Networks of endocardial vessels (20 μm in thickness) with expansions (40-100 μm in diameter) were visualized; they were movable on the endocardium of the bovine atrium and ventricle. CLSM showed that (1) rod-shaped nuclei were aligned along the longitudinal direction of the endocardial vessel and (2) there were many cells inside the expansion. TEM on the endocardial vessel revealed that (1) there existed multiple lumens (1-7 μm in diameter) and (2) the extracellular matrices mostly consisted of collagen fibers, which were aligned along the longitudinal direction of the endocardial vessel or were locally organized in reticular structures. We investigated the endocardial circulatory system in bovine cardiac chambers and its ultrastructures, such as nucleic distributions, microlumens, and collagenous extracellular matrices. Copyright © 2011 S. Karger AG, Basel.

  3. Pressurized Vessel Slurry Pumping

    Energy Technology Data Exchange (ETDEWEB)

    Pound, C.R.

    2001-09-17

    This report summarizes testing of an alternate ''pressurized vessel slurry pumping'' apparatus. The principle is similar to rural domestic water systems and ''acid eggs'' used in chemical laboratories in that material is extruded by displacement with compressed air.

  4. Key advances in the clinical approach to ANCA-associated vasculitis.

    Science.gov (United States)

    Kallenberg, Cees G M

    2014-08-01

    The updated nomenclature for vasculitis defines this varied group of disorders by aetiology, specific features of pathogenesis and clinical symptoms; diagnostic and classification criteria for clinical practice are in development. Here, I review some important advances in the management of vasculitis within the category of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which encompasses microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The clinical approach to the management of the patient with AAV should include testing for ANCA specificity; proteinase 3 (PR3)-specific ANCAs are most often associated with GPA, whereas myeloperoxidase (MPO)-ANCAs are usually associated with MPA. Also important to the management of AAV is an assessment of the disease stage and severity, to enable tailored treatment based on an algorithm derived from controlled-trial data. Remaining questions pertain to the dosage and duration of corticosteroid treatment, the selection of patients for, and duration of, maintenance treatment after induction of remission, and the identification of safer and more effective therapies than are currently in use. Outcome measures should assess not only disease activity, but also damage and quality of life. Infections, cardiovascular events and malignancies also contribute to outcome, and their prevention should therefore be part of the clinical approach to managing patients with AAV.

  5. [Seronegative leukocytoclastic vasculitis preceding the onset of disseminated systemic lupus erythematosus].

    Science.gov (United States)

    Chevalier, X; Belmatoug, N; Kahn, M F

    1990-11-01

    We encountered 4 patients (3 women, 1 man) with cutaneous vasculitis: three have a delayed pressure urticaria, two a vascular purpura with which a mixt cryoglobulinemia. Histology show a leukocytoclastic vasculitis. Initially all studies for lupus erythematosus were negative. However, after 3 to 10 years of follow-up, the 4 patients developed clinical, serological and histological features of systemic lupus erythematosus meeting four or more criteria of the American Rheumatism Association for the diagnosis of SLE. In one case there is a moderate renal disease. In three others cases there are a severe visceral injury: one with aseptic valvula's injury treated by surgery, another who died from a septicemic incident. The last who died from a neurological complication of systemic lupus. During the isolated cutaneous vasculitis phasis antinuclear antibodies and antibodies to double stranded DNA were all negative. At the time of SLE's diagnosis anti-DNA antibodies were present with or without ANA. In all four cases hypocomplementaemia was not initially seen which distinguish these cases from others previously reported in the literature. The syndrome recognized in these patients may constitute an "ante" serological and clinical phasis of SLE.

  6. Necrotizing peripheral vasculitis/vasculopathy following the use of cocaine laced with levamisole.

    Science.gov (United States)

    Farmer, Russell W; Malhotra, Paula S; Mays, Michael P; Egger, Michael E; Smith, Jason W; Jortani, Saeed A; Spiller, Henry; Bosse, George M; Callen, Jeffrey P; Franklin, Glen A

    2012-01-01

    The objective of this study was to describe a novel presentation of peripheral vasculitis associated with levamisole-adulterated cocaine. Cocaine abuse is widespread in the United States with 5.3 million people using cocaine in 2008. Over the past decade, drug enforcement officials have noticed the presence of levamisole in confiscated cocaine samples as an adulterant. Known side effects of cocaine-related levamisole ingestion have included agranulocytosis and a cutaneous acral purpura that is histopathologically characterized by a mixture of inflammation (vasculitis) and occlusion (vasculopathy). A 54-year-old man who nasally ingested cocaine laced with levamisole developed widespread necrotic/purpuric skin lesions on approximately 20% of his body with an acral accentuation. These lesions were complicated by multiple areas of sloughing and necrosis. He was initially treated with topical silver sulfadiazine dressing changes but progressed to require debridement and split-thickness skin grafting. Peripheral vasculitis/vasculopathy with severe necrosis resembling Coumadin necrosis is a relatively recently recognized sequelae from levamisole-adulterated cocaine use.

  7. Phospholipid Syndrome and Vasculitis as a presentation of Systemic Lupus Erythematosus. Case report.

    Directory of Open Access Journals (Sweden)

    Sila Castellón Mortera

    2013-09-01

    Full Text Available The systemic Lupus Erythematosus is presented, generally, as a poli articular syndrome, with a long period of fever nephritico or nephrotico; other clinical ways are: neuropsychiatry, vasculitis, etc. They appeared in a progressive manner; but in rare cases as a sickness debutant. It has not being reported in Sancti Spiritus Province patients in which matches the debut of the systemic Lupus Erythematosus with the manifestations of phospholipid syndrome. A Woman with 24 years of age is hospitalized having vasculitis, articular pains, thrombose in her right foot, detecting anticoagulante lupico and possitive Rematoideo factor with periferic pattern diffused in the Inmunoelectroforesis. 5 years later was hospitalized again with poliserositis. She had a positive evolution with a dose in a month of Intacglobin and anticoagulante treatment. Two years later she was hospitalized with articular pains proving she had livedo reticular on her left knee and Raynaud phenomenon on her foot. Beta Prebeta Index and high triglycerides. Lupico anticoagulant positive again. A treatment with Intacglobin and Prednisona was given to the patient with a better clinic without being hospitalized again. There is no evidence (at 17 years of age of a sickness debut of renal dissorder. It is about a Systemic Lupus Eritematoso which debut was a vasculitis and a Phospholipid Syndrome associated.

  8. Post-transplant immune complex nephritis in a patient with systemic lupus erythematosus associated with ANCA vasculitis.

    Science.gov (United States)

    Sanchez, Carlos; Rebolledo, Alejandra; Gahona, Junior; Rojas, Mauricio; Jiménez, Raquel; Bojórquez, Aurora

    2017-05-01

    Nearly 20% of SLE corresponds to the pediatric population, and 75% of them have kidney involvement representing an important etiology of chronic kidney disease. A correlation between SLE and ANCA-associated vasculitis has been identified as an overlapping syndrome. Kidney allograft recurrence is rare in SLE when disease control is achieved and with nowadays immunosuppression treatment. Histologic transformation is unusual, especially when there are negative serologic markers and no immune complex deposition reported in native kidneys. A 17-year-old female with crescentic glomerulonephritis, p-ANCA-positive antibodies with pauci-immune pattern in kidney biopsy develops end-stage renal disease requiring hemodialysis. Deceased donor kidney transplant was performed receiving triple immunosuppression thereafter. Thirteen months later serum creatinine rises without evidence of infection, urinary obstruction, or clinical and serologic disease relapse. Allograft biopsy reports mesangial proliferation and "full-house" immunofluorescence. The role of ANCA in SLE physiopathology is controversial, and its relation with lupus nephritis is also discordant. ANCA could represent an important factor in the heterogeneity of systemic lupus erythematosus and lupus nephritis. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  9. Gastric presentation (vasculitis) mimics a gastric cancer as initial symptom in granulomatosis with polyangiitis: a case report and review of the literature.

    Science.gov (United States)

    Zheng, Zhaohui; Ding, Jin; Li, Xueyi; Wu, Zhenbiao

    2015-11-01

    Granulomatosis with polyangiitis (GPA), formerly called Wegener's Granulomatosis, is characterized by necrotizing granulomatous inflammation and belongs to the family of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. The main clinical symptoms of GPA are vasculitis primarily involving upper and lower respiratory tracts, as well as kidneys. Gastrointestinal manifestations of GPA are less common (0-20 %), with gastric presentation mimicking a gastric cancer as an initial symptom. This is a descriptive case report of one patient, together with systematic review of the literature. We described a 31-year-old Chinese woman who presented with complaints of abdominal distention, anorexia for 2 months. Gastroscopy was carried out for three times, and stomach cancer was suspected. However, histopathology of gastric biopsy revealed a chronic inflammation with mucosal ulceration, frequent neutrophils and lymphocytes infiltration, and local granulomatous formation, whereas no sign of stomach carcinoma was observed. In view of the positive cANCA test, a diagnosis of GPA was considered. From the onset of the GPA in the patients, no other organs have been involved in the disease. The patient was successfully treated with corticosteroids and cyclophosphamide. As shown in the report, patients who present only with gastrointestinal manifestations represent challenges to diagnosis. ANCA testing can serve as a decisive diagnostic tool. Although uncommon, GI involvement may be a major feature in GPA, sometimes presenting as gastric tumor-like lesions. Diagnosis should be considered in patients presenting with GI symptoms accompanied by evidence of systemic vasculitis, and ANCA test should be used as a diagnostic measurement to clarify differential diagnosis.

  10. Neuroradiologic picture of cerebral vasculitis in rheumatoid arthritis.

    Science.gov (United States)

    Paci, R; Giuffrida, C M; Marangolo, M; Ventura, F; Di Paola, F

    1983-01-01

    The central nervous system is rarely involved in rheumatoid arthritis. In this paper the authors report a case of a 50-year-old woman who had rheumatoid arthritis for about 6 years. A month before admission she presented a symptomatology resembling an expansive intracranial process. Angiography revealed cerebral arteritis and CT showed areas of hypodensity with marked contrast enhancement.

  11. Hawaii Abandoned Vessel Inventory, Kauai

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Kauai. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  12. CNMI Abandoned Vessel Inventory, Tinian

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Tinian. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  13. Puerto Rico Abandoned Vessel Inventory

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Puerto Rico. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral...

  14. American Samoa Abandoned Vessel Inventory

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for American Samoa. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral...

  15. Hawaii Abandoned Vessel Inventory, Oahu

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Oahu, Hawaii. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral...

  16. Hawaii Abandoned Vessel Inventory, Molokai

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Molokai, Hawaii. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral...

  17. CNMI Abandoned Vessel Inventory, Rota

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Rota. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  18. Hawaii Abandoned Vessel Inventory, Lanai

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Lanai. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  19. For-Hire Vessel Directory

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Vessel Directory is maintained as the sample frame for the For-Hire Survey. I contains data on for-hire vessels on the Atlantic and Gulf coasts. Data include...

  20. CNMI Abandoned Vessel Inventory, Saipan

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Saipan. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  1. Hawaii Abandoned Vessel Inventory, Maui

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — NOAA Abandoned Vessel Project Data for Maui. Abandoned vessels pose a significant threat to the NOAA Trust resources through physical destruction of coral habitats...

  2. Vessels in Transit - Web Tool

    Data.gov (United States)

    Department of Transportation — A web tool that provides real-time information on vessels transiting the Saint Lawrence Seaway. Visitors may sort by order of turn, vessel name, or last location in...

  3. Behcet's Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment

    Science.gov (United States)

    Owlia, M. B.; Mehrpoor, G.

    2012-01-01

    Behcet's disease (BD) is the only systemic vasculitis involving both arteries and vein in any sizes. It frequently encounters in rheumatology clinics. It has some major morbidities and even fatal outcomes in some cases. The aim of this paper is to analyze the main concepts on pathophysiology and treatment options in BD, focusing on cardiovascular aspects, thrombosis, and potential future treatment. PMID:22530146

  4. Pressure vessel design manual

    Energy Technology Data Exchange (ETDEWEB)

    Moss, D.R.

    1987-01-01

    The first section of the book covers types of loadings, failures, and stress theories, and how they apply to pressure vessels. The book delineates the procedures for designing typical components as well as those for designing large openings in cylindrical shells, ring girders, davits, platforms, bins and elevated tanks. The techniques for designing conical transitions, cone-cylinder intersections, intermediate heads, flat heads, and spherically dished covers are also described. The book covers the design of vessel supports subject to wind and seismic loads and one section is devoted to the five major ways of analyzing loads on shells and heads. Each procedure is detailed enough to size all welds, bolts, and plate thicknesses and to determine actual stresses.

  5. New research vessels

    Science.gov (United States)

    1984-04-01

    Two “new” ocean-going research vessels operated by the Scripps Institution of Oceanography and the National Science Foundation (NSF) will soon begin full-time scientific duties off the coast of California and in the Antarctic, respectively. The 37.5-m Scripps vessel, named Robert Gordon Sprout in honor of the ex-president of the University of California, replaces the smaller ship Ellen B. Scripps, which had served the institution since 1965. The new ship is a slightly modified Gulf Coast workboat. Under the name of Midnight Alaskan, it had been used for high-resolution geophysical surveys in American and Latin American waters by such firms as Arco Oil & Gas, Exxon, Pennzoil, and Racal-Decca before its purchase by Scripps from a Lousiana chartering firm last summer.

  6. Very Versatile Vessel

    Science.gov (United States)

    2009-09-01

    data. This source provides information on aluminum hydrofoil vessels without the added weight of foil structures. The composite armor around the...seating compartment. The sides should also limit wave splash on the deck. The freeboard should contribute reserve buoyancy , increasing large-angle and...Resistance, Powering, and Propulsion Savitsky’s Method Since model testing data or other reliable performance data was unavailable for the proposed

  7. CLINICAL AND EPIDEMIOLOGICAL CHARACTERISTICS OF KAWASAKI DISEASE WITH SPECIAL REFERENCE TO CARDIAC INVOLVEMENT

    Directory of Open Access Journals (Sweden)

    Jayanta Kumar

    2016-01-01

    Full Text Available BACKGROUND Kawasaki disease (KD is an acute medium vessel vasculitis with multisystem involvement especially in young children of unknown etiology and present as acute febrile illness. It is the leading cause of acquired heart disease in children across the globe. OBJECTIVES We aimed to evaluate the epidemiological characteristics, clinical manifestations and cardiac involvement of KD in children residing in Tripura, a North Eastern State of India. MATERIALS AND METHODS In this retrospective study, we reviewed the medical records of all children with KD who had been admitted in Pediatric ward of Tripura Medical College & DR BRAM Teaching Hospital, Agartala from March 2007 to February 2015. Data regarding clinical, epidemiological characteristics, Cardiac involvement, laboratory parameter, management, and the outcome of disease for each patient were obtained. The patients were divided into cardiac and non-cardiac groups based on echocardiographic results. RESULTS In total, 30 patients with KD (18boys and 12 girls were enrolled in this study. The male to female ratio was 1.5:1. The median age at diagnosis was 15 months, and the diagnosis was made after a mean of 8.1 days of fever. A seasonal peak during the winter-spring months was observed. 23 (76.6% had classical presentation of KD. Fever, polymorphs, skin rash, conjunctivitis, changes in the oropharynx were the most common manifestations. Cardiac involvement was detected in 7(23.3%%, with coronary artery abnormalities (CAA and 26(86.5% patient showed ECG abnormality. Patients were treated with immunoglobulin and aspirin. The CAA regressed in all patients but one persisted even after 12 months. CONCLUSION Kawasaki disease is not rare in Tripura. The age, gender distribution and clinical findings are similar to that of other reports. Patients with cardiac abnormalities had delayed treatment and prolonged hospital stays

  8. Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

    LENUS (Irish Health Repository)

    Little, Mark A

    2012-01-01

    Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3) antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener\\'s granulomatosis). Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻\\/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17%) more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

  9. Anti-proteinase 3 anti-neutrophil cytoplasm autoantibodies recapitulate systemic vasculitis in mice with a humanized immune system.

    Directory of Open Access Journals (Sweden)

    Mark A Little

    Full Text Available Evidence is lacking for direct pathogenicity of human anti-proteinase-3 (PR3 antibodies in development of systemic vasculitis and granulomatosis with polyangiitis (GPA, Wegener's granulomatosis. Progress in study of these antibodies in rodents has been hampered by lack of PR3 expression on murine neutrophils, and by different Fc-receptor affinities for IgG across species. Therefore, we tested whether human anti-PR3 antibodies can induce acute vasculitis in mice with a human immune system. Chimeric mice were generated by injecting human haematopoietic stem cells into irradiated NOD-scid-IL2Rγ⁻/⁻ mice. Matched chimera mice were treated with human IgG from patients with: anti-PR3 positive renal and lung vasculitis; patients with non-vasculitic renal disease; or healthy controls. Six-days later, 39% of anti-PR3 treated mice had haematuria, compared with none of controls. There was punctate bleeding on the surface of lungs of anti-PR3 treated animals, with histological evidence of vasculitis and haemorrhage. Anti-PR3 treated mice had mild pauci-immune proliferative glomerulonephritis, with infiltration of human and mouse leukocytes. In 3 mice (17% more severe glomerular injury was present. There were no glomerular changes in controls. Human IgG from patients with anti-PR3 autoantibodies is therefore pathogenic. This model of anti-PR3 antibody-mediated vasculitis may be useful in dissecting mechanisms of microvascular injury.

  10. [The clinical morphology of cardiac involvement in typhoid in servicemen in Afghanistan].

    Science.gov (United States)

    Bobin, A N

    1994-09-01

    The article is based on the results of studies of protocols of 65 autopsies, 42 medical histories and morphological studies of contemporary records of 47 hearts of the deceased servicemen from the Soviet troops in Afghanistan who died of typhoid. It was found out that myocarditis was developed in 61.9 7.3% of cases regardless of the existence and character of complications. The inalienable features of myocarditis were inflammatory changes (vasculitis, basically mononuclear infiltration of stroma, granulomas), vascular malfunctions (microcirculatory disorders, enhanced permeability of vessels, edema of stroma), dystrophic and necrotic changes in cardiomyocytes. Vasculitis had predominant significance in cardiac morphogenesis. Besides myocardium the inflammatory process had proliferated also on epicardium and mural endocardium, i.e. pancarditis.

  11. 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.

  12. Doppler sonography of extracranial and intracranial vessels in patients with thrombotic stroke

    Directory of Open Access Journals (Sweden)

    Farhad Iranmanesh

    2006-12-01

    Full Text Available INTRODUCTION: The results of intracranial and extracranial vessel color Doppler sonography, which is now an inseparable part of patient evaluation, vary in different studies. The objective of this study was to evaluate the pattern of vascular involvement in thrombotic stroke and its relationship with risk factors of stroke. METHODS: One-hundred patients (45 males and 55 females with thrombotic stroke underwent transcranial sonography and color Doppler sonography of extracranial vessels. The pattern of vascular involvement was analyzed. The risk factors of stroke were also evaluated. RESULTS: Forty-seven percent of the studied individuals had some variations in their color Doppler sonography of extracranial vessels, 27% had changes in the intracranial vessels and 26% showed changes in both. The most frequently involved vessels among the intracranial and extracranial vessels were the middle cerebral artery and the internal carotid artery, respectively. The pattern of vascular involvement was unrelated to hypertension, cigarette smoking, diabetes mellitus, dyslipidemia or history of ischemic heart disease. Extracranial involvement in patients with positive history of MI was more prevalent than in those without such history. CONCLUSIONS: Extracranial vessel involvement in thrombotic stroke was found to be more prevalent than intracranial vessel involvement in the city of Rafsanjan; however, intracranial vessel involvement was more prevalent than in western countries. KEY WORDS: Thrombotic stroke, color Doppler sonography, intracranial vessels, extracranial, cerebrovascular risk factors

  13. Meningeal involvement in Wegener granulomatosis: case report and review of the literature.

    Science.gov (United States)

    Soriano, A; Lo Vullo, M; Casale, M; Quattrocchi, C C; Afeltra, A

    2012-01-01

    Wegener Granulomatosis (WG) is a multisystem autoimmune disorder characterized by necrotizing granulomatous vasculitis that most commonly involves the upper respiratory tract, lungs, and kidneys. The involvement of the central nervous system (CNS) is infrequent and can cause stroke, cranial nerve abnormalities, cerebrovascular events, seizures, and meningeal involvement. Meningeal involvement is rare and may occur due to local vasculitis, directly spread from adjacent disease in the skull base, paranasal or orbital region. We describe the case of a 20-year-old Caucasian man who was diagnosed with sinonasal WG with frontal focal meningeal involvement. A literature review on diagnosis and treatment of meningeal involvement in course of WG was carried out. The importance of an early diagnosis and treatment of localized WG has been emphasized, in order to avoid the progression to a severe form of disease, especially in younger patients and in paucisymptomatic cases.

  14. Vessel Traffic Services.

    Science.gov (United States)

    1982-12-01

    Yorker" articles titled Silent Spring by Rachel Carson in 1963 produced a unifying effect, "the sort of rallying point of the movement to protect the...6232, 92d Cong., 1st. sess., 1971, p. 2. 15. Carson , Rachel L. , The Sea Around Us, New York: Oxford Univesity Press, 195-, p. IV. 16. U.S., Congress...Government Printing Office, 1974. 63. Buhler, L. and Geiger, J., Vessel Traffic Data Extraction MethodoloqX, Silver Spring , Maryland, O6erFae-tns

  15. Vanishing corneal vessels

    Science.gov (United States)

    Nicholson, Luke; Chana, Rupinder

    2013-01-01

    We wish to highlight the importance of acknowledging the accompanying effects of topical phenylephrine drops on the eye other than its intended mydriasis. We reported a case of a 92-year-old woman with a corneal graft who was noted to have superficial corneal vascularisation which was not documented previously. After the instillation of topical tropicamide 1% and phenylephrine 2.5%, for funduscopy, the corneal vascularisation was not visible. When reassessed on another visit, tropicamide had no effect on the vessels and only phenylephrine did. We wish to highlight that when reviewing patients in cornea clinics, instilling phenylephrine prior to being seen may mask important corneal vascularisation. PMID:24121816

  16. OMERACT Endorsement of Patient-reported Outcome Instruments in Antineutrophil Cytoplasmic Antibody-associated Vasculitis.

    Science.gov (United States)

    Robson, Joanna C; Tomasson, Gunnar; Milman, Nataliya; Ashdown, Sue; Boonen, Annelies; Casey, George C; Cronholm, Peter F; Cuthbertson, David; Dawson, Jill; Direskeneli, Haner; Easley, Ebony; Kermani, Tanaz A; Farrar, John T; Gebhart, Don; Lanier, Georgia; Luqmani, Raashid A; Mahr, Alfred; McAlear, Carol A; Peck, Jacqueline; Shea, Beverley; Shea, Judy A; Sreih, Antoine G; Tugwell, Peter S; Merkel, Peter A

    2017-10-01

    The antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are multiorgan diseases. Patients with AAV report impairment in their health-related quality of life (HRQOL) and have different priorities regarding disease assessment compared with physicians. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group previously received endorsement for a core set of domains in AAV. Two approaches to measure patient-reported outcomes (PRO) were presented at OMERACT 2016. A novel 5-step tool was used to facilitate assessment of the instruments by delegates: the OMERACT Filter 2.0 Instrument Selection Algorithm, with a red-amber-green checklist of questions, including (1) good match with domain (face and content validity), (2) feasibility, (3) do numeric scores make sense (construct validity)?, (4) overall ratings of discrimination, and (5) can individual thresholds of meaning be defined? Delegates gave an overall endorsement. Three generic Patient-Reported Outcomes Measurement Information System (PROMIS) instruments (fatigue, physical functioning, and pain interference) and a disease-specific PRO, the AAV-PRO (6 domains related to symptoms and HRQOL), were presented. OMERACT delegates endorsed the use of the PROMIS instruments for fatigue, physical functioning, and pain interference (87.6% overall endorsement) and the disease-specific AAV-PRO instrument (89.4% overall endorsement). The OMERACT Vasculitis Working Group gained endorsement by OMERACT for use of the PROMIS and the AAV-PRO in clinical trials of vasculitis. These instruments are complementary to each other. The PROMIS and the AAV-PRO need further work to assess their utility in longitudinal settings, including their ability to discriminate between treatments of varying efficacy in the setting of a randomized controlled trial.

  17. 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.

  18. 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.

  19. Traumatic endophthalmitis presenting as isolated retinal vasculitis and white-centered hemorrhages: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Nidhi Relhan

    2012-01-01

    Full Text Available The article reports a case and review of the literature of endophthalmitis presenting as isolated retinal vasculitis. A 26-year-old male was observed to have white-centered retinal hemorrhages and retinal vasculitis following an occult scleral perforation. At presentation, the visual acuity was 20/60. With clinical suspicion of early endophthalmitis, he underwent wound exploration, scleral tear repair, vitreous biopsy and administration of intravitreal antibiotics. Microbiology evaluation revealed significant presence of methicillin-resistant coagulase-negative Staphylococcus epidermidis. Final visual acuity improved to 20/20 at 6 weeks postoperatively. Literature search revealed eight similar cases, all of them due to Staphylococcus species. Retinal vasculitis and white-centered retinal hemorrhages can be a presenting sign of early endophthalmitis, especially with non-fulminant pathogens like S. epidermidis.

  20. Hypocomplementemic urticarial vasculitis with Jaccoud's arthropathy and valvular heart disease: case report and review of the literature.

    Science.gov (United States)

    Amano, H; Furuhata, N; Tamura, N; Tokano, Y; Takasaki, Y

    2008-09-01

    We describe a female Japanese patient with concomitant hypocomplementemic urticarial vasculitis, Jaccoud's arthropathy and valvular heart disease. In 1996, she developed arthritis with swelling of both proximal interphalangeal joints and urticarial vasculitis on both arms that was resolved by administration of glucocorticoid (prednisolone 30 mg/day). Tests for antineutrophil cytoplasmic antibodies, antinuclear antibody and rheumatoid factor gave negative results. The findings of a skin biopsy examination were consistent with 'leukocytoclastic vasculitis'. During 10 years of observation, the patient manifested polyarthritis leading to progressive deformity of the joints of the hands and feet (without loss of cartilage or erosion of bone), persistent urticaria exacerbated by cold and accompanied by hypocomplementemia and progressive cardiac valvular disease with mitral valve regurgitation. There are only three reports described previously documenting five patients with this rare combination of manifestations.

  1. Mesenteric vasculitis in adults with Henoch-Schonlein purpura: a not-so-benign condition.

    LENUS (Irish Health Repository)

    Sibartie, V

    2009-02-07

    INTRODUCTION: The gastrointestinal manifestations of Henoch-Schonlein purpura (HSP) are well characterised, but their recognition can be difficult when they occur in isolation. Furthermore, HSP can run a more serious course in adults, compared to children, in whom the disease usually occurs. MATERIALS AND METHODS: We describe two cases that illustrate the challenges of HSP with mesenteric vasculitis and the outcome in adults. CONCLUSION: Although self-limiting in most patients, the outcome of HSP in adults can be far from benign and even fatal.

  2. Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasm in diagnosis of systemic vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Savage, C.O.S.; Winearls, C.G.; Jones, S.; Marshall, P.D.; Lockwood, C.M.

    1987-06-20

    The diagnosis and management of Wegener's granulomatosis and microscopic polyarteritis are complicated by the lack of specific diagnostic tests. The diagnostic performance of a solid-phase radioimmunoassay, which detects the autoantibodies against neutrophil cytoplasm present in these disorders, was assessed in a prospective study of patients with suspected vasculitis and/or rapidly progressive nephritis. The assay had a sensitivity and specificity of 96% when carried out in combination with a specific inhibition stage and indirect immunofluorescence staining of alcohol-fixed normal neutrophils.

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

    Science.gov (United States)

    Morishita, Kimberly A; Moorthy, Lakshmi N; Lubieniecka, Joanna M; Twilt, Marinka; Yeung, Rae S M; Toth, Mary B; Shenoi, Susan; Ristic, Goran; Nielsen, Susan M; Luqmani, Raashid A; Li, Suzanne C; Lee, Tzielan; Lawson, Erica F; Kostik, Mikhail M; Klein-Gitelman, Marisa; Huber, Adam M; Hersh, Aimee O; Foell, Dirk; Elder, Melissa E; Eberhard, Barbara A; Dancey, Paul; Charuvanij, Sirirat; Benseler, Susanne M; Cabral, David A

    2017-07-01

    To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were 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 (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4-6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9-15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4-6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0-6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not

  4. 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.

  5. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis

    DEFF Research Database (Denmark)

    Basu, Neil; Watts, Richard; Bajema, Ingeborg

    2010-01-01

    The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions, classific......, classification and diagnostic criteria. Therefore, the aim of the present work was to identify deficiencies and provide contemporary points to consider for the development of future definitions and criteria in systemic vasculitis.......The systemic vasculitides are multiorgan diseases where early diagnosis and treatment can significantly improve outcomes. Robust nomenclature reduces diagnostic delay. However, key aspects of current nomenclature are widely perceived to be out of date, these include disease definitions...

  6. Vasculite granulomatosa necrosante isolada de colo uterino associada à infecção por papilomavírus humano Isolated necrotizing granulomatous vasculitis of the uterine cervix associated with human papillomavirus infection

    Directory of Open Access Journals (Sweden)

    Nilton Salles Rosa Neto

    2009-10-01

    Full Text Available Vasculites de órgão único, ou isoladas, já foram descritas em diversos órgãos e seu achado pode ser acidental. Relatamos um caso de vasculite granulomatosa necrosante isolada de colo uterino em uma paciente de meia-idade, previamente hígida, sexualmente ativa, e cuja pesquisa de DNA de papilomavírus humano (Human Papiloma Virus - HPV por captura híbrida foi positiva. Não foi identificado comprometimento sistêmico e, como houve excisão completa da lesão, optou-se pelo acompanhamento clínico. Há poucos relatos, na literatura, de acometimento do trato genital feminino de forma isolada, alguns com presença simultânea de lesões que podem ser causadas pelo HPV, postulando-se uma associação patogênica.Single organ vasculitis (SOV, or isolated vasculitis, has been described in several organs and it can be an accidental finding. We report a case of isolated necrotizing granulomatous vasculitis of the uterine cervix in a middle-aged woman, previously healthy, and sexually active, and whose human papillomavirus (HPV DNA hybrid capture assay was positive. Systemic involvement was not detected and, since the lesion was completely removed, we opted for a clinical follow-up. The literature has very few reports on the isolated involvement of the female genital tract, and some had concomitant lesions that could be caused by the HPV, indicati.

  7. Displasia fibromuscular: um diagnóstico diferencial para as vasculites Fibromuscular dysplasia: a differential diagnosis of vasculitis

    Directory of Open Access Journals (Sweden)

    Thaís de Carvalho Pontes

    2012-02-01

    unknown, despite many theories. A genetic component is suspected to exist, because the pathology affects primarily Caucasians. Association between FMD and the HLA-DRw6 histocompatibility antigen has also been described. The major sites affected are renal, cerebral, carotid, visceral, iliac, subclavian, brachial and popliteal arteries. Clinical manifestations correlate with the affected site, arterial hypertension being a frequent symptom, resulting from the involvement of the renal arteries in 60%-75% of the cases. The diagnosis of FMD is made by histopathology and/or angiography. FMD can manifest as a systemic vascular disease, mimicking vasculitis. This understanding is important because vasculitis and FMD can both have a severe clinical course, but require distinct treatments. The differential diagnosis can be difficult in face of an atypical clinical presentation or lack of histopathologic confirmation. Isolated cases of FMD have been reported mimicking the following conditions: polyarteritis nodosa, Ehlers-Danlos's syndrome, Alport's syndrome, pheochromocytoma, Marfan's syndrome, and Takayasu's arteritis. Rheumatologists should be aware of this differential diagnosis. Treatment of FMD is recommended only in symptomatic cases, and consists in revascularization, which may be either surgical or via percutaneous transluminal angioplasty. In FMD, the effects of corticotherapy can directly and rapidly harm the vascular wall, aggravating the lesions

  8. 46 CFR 289.2 - Vessels included.

    Science.gov (United States)

    2010-10-01

    ... CONSTRUCTION-DIFFERENTIAL SUBSIDY VESSELS, OPERATING-DIFFERENTIAL SUBSIDY VESSELS AND OF VESSELS SOLD OR ADJUSTED UNDER THE MERCHANT SHIP SALES ACT 1946 § 289.2 Vessels included. Vessels subject to the provisions of this part are: (a) All vessels which may in the future be constructed or sold with construction...

  9. Multiscale Vessel-guided Airway Tree Segmentation

    DEFF Research Database (Denmark)

    Lo, Pechin Chien Pau; Sporring, Jon; de Bruijne, Marleen

    2009-01-01

    This paper presents a method for airway tree segmentation that uses a combination of a trained airway appearance model, vessel and airway orientation information, and region growing. The method uses a voxel classification based appearance model, which involves the use of a classifier...... is evaluated within EXACT’09 on a diverse set of CT scans. Results show a favorable combination of a relatively large portion of the tree detected correctly with very few false positives....

  10. Blood flow reprograms lymphatic vessels to blood vessels.

    Science.gov (United States)

    Chen, Chiu-Yu; Bertozzi, Cara; Zou, Zhiying; Yuan, Lijun; Lee, John S; Lu, MinMin; Stachelek, Stan J; Srinivasan, Sathish; Guo, Lili; Vicente, Andres; Vincente, Andres; Mericko, Patricia; Levy, Robert J; Makinen, Taija; Oliver, Guillermo; Kahn, Mark L

    2012-06-01

    Human vascular malformations cause disease as a result of changes in blood flow and vascular hemodynamic forces. Although the genetic mutations that underlie the formation of many human vascular malformations are known, the extent to which abnormal blood flow can subsequently influence the vascular genetic program and natural history is not. Loss of the SH2 domain-containing leukocyte protein of 76 kDa (SLP76) resulted in a vascular malformation that directed blood flow through mesenteric lymphatic vessels after birth in mice. Mesenteric vessels in the position of the congenital lymphatic in mature Slp76-null mice lacked lymphatic identity and expressed a marker of blood vessel identity. Genetic lineage tracing demonstrated that this change in vessel identity was the result of lymphatic endothelial cell reprogramming rather than replacement by blood endothelial cells. Exposure of lymphatic vessels to blood in the absence of significant flow did not alter vessel identity in vivo, but lymphatic endothelial cells exposed to similar levels of shear stress ex vivo rapidly lost expression of PROX1, a lymphatic fate-specifying transcription factor. These findings reveal that blood flow can convert lymphatic vessels to blood vessels, demonstrating that hemodynamic forces may reprogram endothelial and vessel identity in cardiovascular diseases associated with abnormal flow.

  11. Efficacy of TNF-α antagonist and other immunomodulators in the treatment of patients with ophthalmologic manifestations of Behcet's disease and HLA B51 positive vasculitis

    Directory of Open Access Journals (Sweden)

    Zlatanović Gordana

    2012-01-01

    Full Text Available Bacground/Aim. Behcet's disease is genetically conditioned, immune-mediated multisystem occlusive vasculitis of small blood vessels, espesially venules, of unknown etiology. The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. Methods. In this study symptoms and signs of the disease were analyzed both prospectively and retrospectively during the active manifestation of the disease. The diagnosis was reached according to the International Criteria for Behcet's Disease (2006. The treatment effects were evaluated based on the presence of the best corrected visual acuity and the inflammation of the vitreous humour before and after the application of our therapeutic method. The applied therapeutic modality consisted of the primary application of corticosteroid therapy in the active stage of the disease complemented with the choice of drugs from the immunosuppressive group. In this study there drugs were cyclosporine or methotrexate. A treatment refractory patients with poor vision prognosis were treated with a third drug, the biological preparation infliximab, a tumor necrosis factor-alpha (TNF-α antagonist. Results. The mean age of 11 patients with ophthalmologic manifestation of Behcet's disease was 50.6 years. HLA B-5(51 was positive in 81% of the patients while 36% of the patients had positive pathergy test. Changes in affected eyes included vitritis (100%, posterior uveitis (45%, panuveitis (54%, retinal vasculitis (54%, cystoid macular edema (54%, and cystoid degeneration (18%. Increased intraocular pressure was observed in 27% of the patients. There was no statistically significant variation in disease activity parameters in any of the patients (p > 0.05. A statistically significant improvement in visual acuity (p < 0.05 and a high statistically significant decrease of inflammation of the worst affected eyes (p = 0.001 were detected

  12. A case of precocious emphysema and lung cancer in a woman with a history of hypocomplementemic urticarial vasculitis.

    Science.gov (United States)

    Jamison, Suzanna C; Brierre, Stephen; Sweet, Jon; de Boisblanc, Ben

    2008-03-01

    Severe emphysema developed in a white woman with a 26-pack-year history of tobacco use. Serum alpha(1)-antitrypsin levels were normal. A history of autoimmune hemolytic anemia, angioedema, low complement, and recurrent urticaria prompted an immunologic workup that ultimately led to a diagnosis of hypocomplementemic urticarial vasculitis syndrome. Treatment with oral prednisone and inhaled bronchodilators improved symptoms, but 4 months after diagnosis non-small cell lung cancer was discovered and she ultimately died. Hypocomplementemic urticarial vasculitis is an uncommon cause of precocious emphysema and has not previously been reported in a patient with bronchogenic carcinoma.

  13. Earlywood vessels of Castanea sativa record temperature before their formation.

    Science.gov (United States)

    Fonti, Patrick; Solomonoff, Natalie; García-González, Ignacio

    2007-01-01

    The aim of this study was to identify the climatic signal contained in the earlywood vessel size of the ring-porous chestnut (Castanea sativa) and the physiological processes involved in the underlying mechanisms. In order to assign the encoded signal to a specific physiological process, bud phenology and vessel formation were monitored along an elevation transect and chronologies of the size of the first row of earlywood vessels were retrospectively correlated with 40 yr of early spring temperatures. The first vessels appeared in late April to early May, after encoding both a negative temperature signal in February-March (during tree quiescence) and a positive temperature signal in early April (at the time of resumption of shoot growth). We hypothesize that February and March temperatures affect cambial sensitivity to auxin, preconditioning tree responses later in the season. Furthermore, April temperature is related to tree activation whereby new hormone production fosters vessel expansion.

  14. Púrpura de Henoch-Schönlein com acometimento incomum de face Unusual face involvement in Henoch-Schönlein purpura

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    Carolina Bassoli de Azevedo

    2009-12-01

    Full Text Available A Púrpura de Henoch-Shönlein é uma vasculite de causa idiopática, que se caracteriza pelo depósito predominante de IgA na parede dos pequenos vasos, envolvendo tipicamente pele, intestino, articulações e glomérulo renal. O acometimento cutâneo ocorre principalmente em membros inferiores e região glútea, sendo raramente encontrado em face e membros superiores. Relatamos o caso de uma paciente do sexo feminino, com seis anos de idade, que apresentava lesões púrpuricas em região auricular, periorbital, malar, mentoniana e membros superiores, além de artralgia e volvo intestinal. Após tratamento cirúrgico e pulsoterapia com glicocorticoide, houve regressão dos sintomas, sem maiores complicações.Henoch-Schönlein Purpura is an idiopathic vasculitis characterized by deposits of immunoglobulin, mainly IgA, on the walls of small vessels, typically involving the skin, gut, joints, and renal glomeruli. Cutaneous involvement affects specially the lower limbs and buttocks, and it is seldom found on the face and upper limbs. We report the case of a 6-year old girl with purpuric lesions over the auricular, periorbital, malar, and mentonian regions and the upper limbs, arthralgia, and intestinal torsion. After surgical treatment and pulse therapy with glucocorticoids, her symptoms subsided without further complications.

  15. The role of biopsies and autopsies in the diagnosis of cognitive impairment, with emphasis on small vessel diseases: A critical appraisal enriched by personal experience

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    Leila Chimelli

    Full Text Available ABSTRACT. Acquired and hereditary microangiopathies cause cerebral small vessel diseases (CSVD that impair cognition. The most frequent is primary angiitis of the CNS (PACNS, whose diagnosis remains challenging, requiring a multidisciplinary approach. Secondary vasculitis, CADASIL, miscellaneous microangiopathies and lymphomas, also cause cognitive impairment. Despite the fact that the need for biopsy has decreased in the era of new neuroimaging methods, biopsies that include small leptomeningeal and parenchymal arterial vessels still remain the gold standard to diagnose PACNS and other CSVD, and to exclude mimics such as infections and malignancies. New approaches for pathological consequences relevant to vascular cognitive impairment such as silent brain lesions, microinfarcts, microbleeds and subtle loss of microstructural integrity, may be detected in autopsies. This article addresses the role of biopsies and autopsies for the diagnosis of cognitive impairment related to small vessel diseases or other inflammatory/ischemic processes, and presents a critical appraisal based on personal experience.

  16. The vessel fluence; Fluence cuve

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    2001-07-01

    This book presents the proceedings of the technical meeting on the reactors vessels fluence. They are grouped in eight sessions: the industrial context and the stakes of the vessels control; the organization and the methodology for the fluence computation; the concerned physical properties; the reference computation methods; the fluence monitoring in an industrial context; vessels monitoring under irradiation; others methods in the world; the research and development programs. (A.L.B.)

  17. Systemic lupus erythematosus and ocular involvement: an overview.

    Science.gov (United States)

    Dammacco, Rosanna

    2017-12-14

    Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control. Keratoconjunctivitis sicca or secondary Sjögren's syndrome is the most frequent ophthalmic manifestation of SLE. According to its variable severity, lubricating tear drops may be sufficient in mild cases, whereas cyclosporine-A ophthalmic solution, glucocorticoids (GCs), methotrexate, and/or other immunosuppressive drugs may be required in the more severe cases. Partial occlusion of the lacrimal punctum by thermal cautery is rarely applied. Although uncommon, episcleritis and scleritis can sometimes be detected as an initial finding of SLE and reveal themselves as moderate to intense ocular pain, redness, blurred vision, and lacrimation. Unilateral or more often bilateral retinopathy is responsible for visual loss of variable severity and is ascribed to vasculitis of the retinal capillaries and arterioles. In addition to the combined treatment suitable for all patients with active SLE, intravitreal bevacizumab should be considered in cases of severe vaso-occlusive retinopathy and laser photocoagulation in cases of neovascularization. Purtscher-like retinopathy is likely ascribable to the formation of microemboli that results in retinal vascular occlusion and microvascular infarcts. Choroidal disease is characterized by monolateral or bilateral blurred vision. Because of the choroidal effusion, retinal

  18. Análisis Histórico de la Vasculitis. Clasificación y propuestas para un entendimiento.

    Directory of Open Access Journals (Sweden)

    Antonio Iglesias Gamarra

    2002-04-01

    Full Text Available

    Apartir de 1978, siendo residente de Reumatología iniciamos el estudio de las vasculitis en el Hospital San Juan de Dios de Bogotá. Se escribió el primer texto de vasculitis necrotizante que ganó el premio Salvat en 1980 y se publicó en 1982: Este libro tenía como objeto informar el tema a la comunidad médica.

    Durante mi estadía en Barranquilla, se realizó una descripción de 9 pacientes con los doctores Eduardo Egea, Gloria Garavito y Silvio Severini sobre vasculitis linfomonocítica. Previamente se conocían algunos informes en la literatura médica como el realizado por Mc’Duffie fundador del Laboratorio de la Clínica Mayo en Rochester y Jorge Maldonado quienes describieron una vasculitis asociada a urticaria y se documentó una precipitina C1q, pero que no reunía los criterios de lupus eritematoso sistémico.

    Otra publicación era la de Soter quien describió dos tipos de vasculitis, la leucocitoclastica y la linfomonocitica. Con estos estudios previos, Iglesias y col estudiamos 9 pacientes que consultaron por urticaria, lesiones eritematosas, papulas, etc. Las lesiones de urticaria tenían una característica importante y era que permanecían por más de 24 horas en el sitio de la piel comprometida, eran de tipo urente y al tomar una biopsia de esta lesión se observaba un infiltrado linfoóplasmocitario; posiblemente su patogénesis está relacionada con un mecanismo de tipo celular y participan una serie de proteínas (como integrinas, selectinas y moléculas de adhesión.

    Se documenta que las vasculitis linfomonocíticas se asocian a algunos medicamentos, procesos infecciosos, o son secundarias a algunas enfermedades del tejido conjuntivo como el lupus y el síndrome de Sjögren primario.

    Desde hace más o menos cinco años estamos estudiando en las unidades de reumatología, dermatología y patología del Hospital San Juan de Dios, a los pacientes con lesiones nodulares, paniculitis y eritema

  19. Vessel segmentation in screening mammograms

    Science.gov (United States)

    Mordang, J. J.; Karssemeijer, N.

    2015-03-01

    Blood vessels are a major cause of false positives in computer aided detection systems for the detection of breast cancer. Therefore, the purpose of this study is to construct a framework for the segmentation of blood vessels in screening mammograms. The proposed framework is based on supervised learning using a cascade classifier. This cascade classifier consists of several stages where in each stage a GentleBoost classifier is trained on Haar-like features. A total of 30 cases were included in this study. In each image, vessel pixels were annotated by selecting pixels on the centerline of the vessel, control samples were taken by annotating a region without any visible vascular structures. This resulted in a total of 31,000 pixels marked as vascular and over 4 million control pixels. After training, the classifier assigns a vesselness likelihood to the pixels. The proposed framework was compared to three other vessel enhancing methods, i) a vesselness filter, ii) a gaussian derivative filter, and iii) a tubeness filter. The methods were compared in terms of area under the receiver operating characteristics curves, the Az values. The Az value of the cascade approach is 0:85. This is superior to the vesselness, Gaussian, and tubeness methods, with Az values of 0:77, 0:81, and 0:78, respectively. From these results, it can be concluded that our proposed framework is a promising method for the detection of vessels in screening mammograms.

  20. Americium behaviour in plastic vessels

    Energy Technology Data Exchange (ETDEWEB)

    Legarda, F.; Herranz, M. [Departamento de Ingenieria Nuclear y Mecanica de Fluidos, Escuela Tecnica Superior de Ingenieria de Bilbao, Universidad del Pais Vasco (UPV/EHU), Alameda de Urquijo s/n, 48013 Bilbao (Spain); Idoeta, R., E-mail: raquel.idoeta@ehu.e [Departamento de Ingenieria Nuclear y Mecanica de Fluidos, Escuela Tecnica Superior de Ingenieria de Bilbao, Universidad del Pais Vasco (UPV/EHU), Alameda de Urquijo s/n, 48013 Bilbao (Spain); Abelairas, A. [Departamento de Ingenieria Nuclear y Mecanica de Fluidos, Escuela Tecnica Superior de Ingenieria de Bilbao, Universidad del Pais Vasco (UPV/EHU), Alameda de Urquijo s/n, 48013 Bilbao (Spain)

    2010-07-15

    The adsorption of {sup 241}Am dissolved in water in different plastic storage vessels was determined. Three different plastics were investigated with natural and distilled waters and the retention of {sup 241}Am by these plastics was studied. The same was done by varying vessel agitation time, vessel agitation speed, surface/volume ratio of water in the vessels and water pH. Adsorptions were measured to be between 0% and 70%. The adsorption of {sup 241}Am is minimized with no water agitation, with PET or PVC plastics, and by water acidification.

  1. Fibrosis pulmonar asociada a vasculitis con anticuerpos anticitoplasmáticos positivos

    Directory of Open Access Journals (Sweden)

    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.

  2. Necrotizing mycotic vasculitis with cerebral infarction caused by Aspergillus niger in a horse with acute typholocolitis.

    Science.gov (United States)

    Tunev, S S; Ehrhart, E J; Jensen, H E; Foreman, J H; Richter, R A; Messick, J B

    1999-07-01

    An 18-year-old Morgan mare was presented to the Veterinary Medical Teaching Hospital, University of Illinois, with a 10-day history of watery diarrhea, depression, and dysphagia. On admission, the animal was severely dehydrated, depressed, and unable to swallow and had no clinical signs of diarrhea. The respiratory and heart rate and body temperature were within normal limits. Following fluid therapy, the mare developed severe watery diarrhea and continued to be depressed, incoordinated, and dysphagic. The animal died on the fourth day after admission and was sent to the Laboratories of Veterinary Diagnostic Medicine for necropsy. Gross postmortem findings were consistent with an acute cerebral infarction in the right cerebral hemisphere, an acute necrotizing typhlocolitis, multifocal petechial and ecchymotic hemorrhages, enlarged and congested pars intermedia of the pituitary gland, and marked bilateral adrenocortical hyperplasia with multifocal areas of necrosis and hemorrhage. Histologic evaluation of the affected brain demonstrated an area of coagulative necrosis of the gray matter, with hemorrhage, vasculitis, and thrombosis. There were many fungal hyphae 3.5-6.0 microm, pale basophilic, septate, and occasionally branching at 45 degrees present in the arterial walls and throughout the necrotic tissue. Immunohistochemical analysis revealed Aspergillus niger as the etiologic agent responsible for the mycotic vasculitis and infarction in the brain. Bacteria culture and immunohistochemical staining of the colon and cecum failed to demonstrate specific pathogens.

  3. De Novo PTEN Mutation in a Young Boy with Cutaneous Vasculitis

    Directory of Open Access Journals (Sweden)

    Angela Mauro

    2017-01-01

    Full Text Available Phosphatase and tensin homolog (PTEN is the protein encoded by the PTEN gene (10q23.3. PTEN mutations are related to a variety of rare diseases referred to collectively as PTEN hamartoma tumor syndromes (PHTS, which include Cowden Syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus Syndrome, and Proteus-like syndrome. These diseases are associated with an increased risk of malignancy and for this reason an accurate and early diagnosis is essential in order to institute cancer surveillance. PTEN is a regulator of growth and homeostasis in immune system cells, although there are limited data describing immune dysregulation caused by PTEN mutations. We describe a case of PHTS syndrome caused by a de novo mutation in PTEN detected using a targeted next generation sequencing (NGS gene panel which was instigated for workup of cutaneous vasculitis. We highlight the diagnostic utility of this approach and that mutations in PTEN may be associated with immune-dysregulatory features such as vasculitis in young children.

  4. Anti-Plasminogen Antibodies Compromise Fibrinolysis and Associate with Renal Histology in ANCA-Associated Vasculitis

    Science.gov (United States)

    Berden, Annelies E.; Nolan, Sarah L.; Morris, Hannah L.; Bertina, Rogier M.; Erasmus, Dianhdra D.; Hagen, E. Christiaan; Hayes, Donal P.; van Tilburg, Nico H.; Bruijn, Jan A.; Savage, Caroline O.S.; Bajema, Ingeborg M.

    2010-01-01

    Antibodies recognizing plasminogen, a key component of the fibrinolytic system, associate with venous thrombotic events in PR3-ANCA vasculitis. Here, we investigated the prevalence and function of anti-plasminogen antibodies in independent UK and Dutch cohorts of patients with ANCA-associated vasculitis (AAV). We screened Ig isolated from patients (AAV-IgG) and healthy controls by ELISA. Eighteen of 74 (24%) UK and 10/38 (26%) Dutch patients with AAV had anti-plasminogen antibodies compared with 0/50 and 1/61 (2%) of controls. We detected anti-plasminogen antibodies in both PR3-ANCA– and MPO-ANCA–positive patients. In addition, we identified anti-tissue plasminogen activator (tPA) antibodies in 13/74 (18%) patients, and these antibodies were more common among patients with anti-plasminogen antibodies (P = 0.011). Eighteen of 74 AAV-IgG (but no control IgG) retarded fibrinolysis in vitro, and this associated with anti-plasminogen and/or anti-tPA antibody positivity. Only 4/18 AAV-IgG retarded fibrinolysis without harboring these antibodies; dual-positive samples retarded fibrinolysis to the greatest extent. Patients with anti-plasminogen antibodies had significantly higher percentages of glomeruli with fibrinoid necrosis (P fibrinolysis in vitro. Seropositivity for anti-plasminogen antibodies correlates with hallmark renal histologic lesions and reduced renal function. Conceivably, therapies that enhance fibrinolysis might benefit a subset of AAV patients. PMID:20847144

  5. Mitoxantrone Therapy for Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Cerebral Vasculitis

    Directory of Open Access Journals (Sweden)

    Hélène Massé

    2009-01-01

    Full Text Available Purpose. To report favorable outcome of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE associated with cerebral vasculitis after treatment with immunosuppressive therapy by mitoxantrone. Design. Single case report. Method. A 22-year-old man presented with acute isolated bilateral loss of vision revealing APMPPE. Corticosteroid therapy was initiated and visual acuity gradually improved. Seventeen days later, visual function deteriorated again, associated with flu-like syndrome and severe headaches. A relapse of APMPPE was diagnosed, complicated with lymphocytic meningitis and cerebral ischemia. Intravenous therapy with mitoxantrone was performed in combination with methylprednisolone. Results. Headaches disappeared in a few days whereas visual acuity gradually improved and stabilized at 20/40 in the right eye and 20/32 in the left eye. No adverse event was observed. Clinical improvement was confirmed by magnetic resonance imaging. Conclusion. Cerebral vasculitis is the most severe complication of the extraocular manifestations of APMPEE. This diagnosis should be evoked when severe headaches or behavior disorder are associated with APMPEE.

  6. Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis*

    Science.gov (United States)

    Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva

    2013-01-01

    BACKGROUND It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS Patients with active chronic urticaria had the highest serum levels of D-dimer (purticarial vasculitis. PMID:23793207

  7. Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis.

    Science.gov (United States)

    Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; Reis, Vitor Manoel Silva dos

    2013-01-01

    It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. Patients with active chronic urticaria had the highest serum levels of D-dimer (purticarial vasculitis.

  8. Initial digital vasculitis in a large multicenter cohort of childhood-onset systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Ana Paula Sakamoto

    Full Text Available Abstract Objectives: To assess clinical digital vasculitis (DV as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE within a large population. Methods: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results: DV was observed in 25/852 (3% cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%, periungual infarction in 7 (28%, tip finger ulceration in 4 (16%, painful nodules in 1 (4% and gangrene in 1 (4%. A poor outcome, with digital resorption, occurred in 5 (20%. Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008, discoid rash (16% vs. 4%, p = 0.017, photosensitivity (76% vs. 45%, p = 0.002 and other cutaneous vasculitides (80% vs. 19%, p 0.05. SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28 vs. 14 (0-58, p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014 was significantly lower in the DV group. Conclusion: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.

  9. Hemorrhagic occlusive retinal vasculitis after inadvertent intraocular perforation with gentamycin injection.

    Science.gov (United States)

    Querques, Lea; Miserocchi, Elisabetta; Modorati, Giulio; Querques, Giuseppe; Bandello, Francesco

    2017-03-10

    To report a case of hemorrhagic occlusive retinal vasculitis (HORV) secondary to intraocular toxicity due to inadvertent intraocular injection of gentamycin. A 21-year-old woman was referred to our department because of severe ocular pain and sudden visual loss in her left eye after she received a subconjunctival gentamycin injection for chronic infectious keratitis. At presentation, best-corrected visual acuity was 20/20 in the right eye and counting fingers in the left eye. Fundus examination showed diffuse intraretinal and perivascular hemorrhages, vascular cuffing, white-centered hemorrhages, and diffuse retinal edema. Fluorescein angiography confirmed occlusive retinal vasculitis with capillary nonperfusion and spectral-domain optical coherence tomography revealed ischemic macular edema. The clinical diagnosis was compatible with HORV secondary to retinal toxicity due to high dose of intraocular gentamycin. We report a case of HORV secondary to inadvertent subconjunctival gentamycin injection. Ocular perforation and high dose of intravitreal gentamycin administration should be considered as a potential cause of HORV following subconjunctival injection.

  10. Necrotic erythema nodosum leprosum with oral mucosal involvement.

    Science.gov (United States)

    Swain, J P; Soud, A; Agarwal, S K

    2008-01-01

    Leprosy is a chronic, infectious disease with varied clinical presentations. The type 2 lepra reaction / erythema nodosum leprosum (ENL) classically involves extremities and face. Besides to the classical lesions of type 2 lepra reaction sometimes rare findings of vesicular lesions with necroticans may be observed. We are reporting a case of lepromatous leprosy with bullous reaction and necroticans involving usual sites along with the unusual involvement of oral cavity, which on first look mimicked necrotizing vasculitis or other bullous disorders. This manifestation of type 2 lepra reaction in oral mucosa should be kept in mind by the physicians while dealing with a case of type 2 lepra reaction.

  11. Complement regulatory proteins in kidneys of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis.

    Science.gov (United States)

    Cheng, L; Gou, S-J; Qiu, H-Y; Ma, L; Fu, P

    2018-01-01

    The complement system activation is involved in the development of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). The study aimed to investigate the expression of complement regulatory proteins (CRPs) CD46, CD55 and CD59 in kidneys of 51 AVV patients. The expression of CD46, CD55 and CD59 in kidneys was detected by immunohistochemistry and double immunofluorescence staining. The immunohistochemical examination revealed that expression of the three CRPs could be detected in the glomeruli and tubules of both AAV patients and normal controls. The expression levels of the three CRPs in glomeruli of patients with AAV were significantly lower than those of normal controls. The scores of CD46 and CD55 expression in the tubules of AAV patients were significantly lower than those of normal controls, while there was no significant difference between the scores of CD59 expression in tubules of AAV patients and those of normal controls. Among AAV patients, the expression level of CD46 in glomeruli correlated inversely with the proportion of normal glomeruli, while it correlated with tubular atrophy in renal interstitium (r = -0·305, P = 0·026; r = 0·330, P = 0·023, respectively). The expression levels of CD55 and CD59 in glomeruli correlated with the proportion of total crescents (r = 0·384, P = 0·006; r = 0·351, P = 0·011, respectively). Double immunofluorescence staining indicated that all three CRPs were expressed on endothelial cells, podocytes and mesangial cells in glomeruli. The expression levels of the three CRPs were dysregulated in kidneys of patients with AAV. The expression levels of CD46, CD55 and CD59 were associated with the severity of renal injury of AAV patients. © 2017 British Society for Immunology.

  12. Summary of Reported Whale-Vessel Collisions in Alaskan Waters

    Directory of Open Access Journals (Sweden)

    Janet L. Neilson

    2012-01-01

    Full Text Available Here we summarize 108 reported whale-vessel collisions in Alaska from 1978–2011, of which 25 are known to have resulted in the whale's death. We found 89 definite and 19 possible/probable strikes based on standard criteria we created for this study. Most strikes involved humpback whales (86% with six other species documented. Small vessel strikes were most common (<15 m, 60%, but medium (15–79 m, 27% and large (≥80 m, 13% vessels also struck whales. Among the 25 mortalities, vessel length was known in seven cases (190–294 m and vessel speed was known in three cases (12–19 kn. In 36 cases, human injury or property damage resulted from the collision, and at least 15 people were thrown into the water. In 15 cases humpback whales struck anchored or drifting vessels, suggesting the whales did not detect the vessels. Documenting collisions in Alaska will remain challenging due to remoteness and resource limitations. For a better understanding of the factors contributing to lethal collisions, we recommend (1 systematic documentation of collisions, including vessel size and speed; (2 greater efforts to necropsy stranded whales; (3 using experienced teams focused on determining cause of death; (4 using standard criteria for validating collision reports, such as those presented in this paper.

  13. Systematic review of the role of rituximab in treatment of antineutrophil cytoplasmic autoantibody-associated vasculitis, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch–Schönlein purpura, ankylosing spondylitis, and Raynaud's phenomenon

    Directory of Open Access Journals (Sweden)

    Taha R

    2017-12-01

    Full Text Available Rbab Taha,1 Hadeel El-Haddad,1 Abdulqader Almuallim,2 Fatma Alshaiki,3 Elaf Obaid,2 Hani Almoallim1,2,4 1Department of Medicine, Dr Soliman Fakeeh Hospital, Jeddah, 2Department of Medicine, Faculty of Medicine, Umm Al-Qura University, Mecca, 3Department of Medicine, East Jeddah Hospital, Jeddah, 4Rheumatic Diseases, Umm Al-Qura University, Mecca, Saudi Arabia Abstract: Rituximab (RTX is established for the treatment of rheumatoid arthritis. This systematic review of the literature since 2006 summarizes evidence for the use of RTX in the treatment of additional rheumatological diseases: antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV, hepatitis C virus-related cryoglobulinemic vasculitis, Henoch–Schönlein purpura, ankylosing spondylitis, and Raynaud’s phenomenon. Data from randomized controlled trials are available only for AAV, confirming efficacy for remission induction, including in disease resistant to conventional treatment, and maintenance of remission. Further studies are required to confirm optimal maintenance regimens in AAV, important questions needing to be addressed including protocol administration versus treatment in response to clinical relapse and the importance of maintaining B-cell depletion. Sufficient data are available in other diseases to suggest RTX to be useful and that randomized controlled trials should be conducted. Keywords: anti-CD20 monoclonal antibody, anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, refractory ankylosing spondylitis, resistant cryoglobulinemic vasculitis, refractory rheumatological diseases 

  14. HEMOBILIA DUE TO VASCULITIS OF THE GALL-BLADDER IN A PATIENT WITH MIXED CONNECTIVE-TISSUE DISEASE

    NARCIS (Netherlands)

    KUIPERS, EJ; VANLEEUWEN, MA; NIKKELS, PGJ; JAGER, J; VANRIJSWIJK, MH

    A 30-year-old woman with mixed connective tissue disease presented with acute abdominal pain and signs of blood loss. At cholecystectomy she appeared to have lost a considerable amount of blood into the biliary tract due to bleeding of the gall bladder. Microscopic examination showed vasculitis of

  15. Urinary CD4+ Effector Memory T Cells Reflect Renal Disease Activity in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

    NARCIS (Netherlands)

    Abdulahad, Wayel H.; Kallenberg, Cees G. M.; Limburg, Pieter C.; Stegeman, Coen A.

    Objective. Numbers of circulating CD4+ effector memory T cells are proportionally increased in patients with proteinase 3 antineutrophil cytoplasmic antibody-associated vasculitis (AAV) whose disease is in remission and are decreased during active disease, which presumably reflects their migration

  16. Leukocytoclastic vasculitis associated with hairy cell leukemia at diagnosis: a case report and review of the literature.

    Science.gov (United States)

    Broccoli, Alessandro; Gandolfi, Letizia; Pellegrini, Cinzia; Agostinelli, Claudio; Argnani, Lisa; Zinzani, Pier Luigi

    2016-11-11

    Autoimmune manifestations may occur in patients with hairy cell leukemia (HCL), and some rare cases of polyarteritis nodosa and leukocytoclastic vasculitis have been reported. However, data regarding the treatment of these cutaneous manifestations are lacking, given the rarity of the concomitance of HCL and vasculitic syndromes. We present a 37-year-old man with paraneoplastic leukocytoclastic vasculitis complicating newly diagnosed HCL. The vasculitis completely resolved after the first 3 weekly administrations of cladribine, which is regarded as the gold-standard treatment for this disease. The underlying leukemia showed refractoriness to the same agent, thus requiring a second line of treatment. The clinical picture we have observed is of interest for the following reasons: i) it confirms an existing pathogenetic relationship between this lymphoproliferative disorder and its cutaneous manifestations, as suggested by the prompt resolution of the purpuric lesions upon cladribine administration; ii) it indicates that cladribine is an effective treatment for HCL-related paraneoplastic syndromes, including leukocytoclastic vasculitis; iii) the evolution and the outcomes of the paraneoplastic manifestations may be independent of those of the underlying leukemia, which showed less than a partial response to its initial treatment.

  17. Vasculitis Foundation

    Science.gov (United States)

    ... more » View All News Connect with us on social media! Join VF Donate Next Page » Next Page » More common than you think. More serious than you know. Support. Awareness. Education. Become a member today! Our Mission The ...

  18. Rheumatoid Vasculitis

    Science.gov (United States)

    ... as inflammation in the sac surrounding the heart (pericarditis), inflammation in the lining of the lungs (pleuritis), ... of the lining around the heart or lungs (pericarditis or pleurisy/pleuritis). Patients with a history of ...

  19. Bilateral idiopathic retinal vasculitis following coxsackievirus A4 infection: a case report.

    Science.gov (United States)

    Mine, Izumi; Taguchi, Manzo; Sakurai, Yutaka; Takeuchi, Masaru

    2017-07-19

    Coxsackieviruses are members of a group of viruses called the enteroviruses, which may cause respiratory and gastrointestinal symptoms, erythema, meningoencephalitis, myocarditis, pericarditis, and myositis. Unilateral acute idiopathic maculopathy caused by coxsackievirus A16 has been associated with hand, foot, and mouth disease, but only a few reports describe retinitis associated with coxsackievirus serotype B3 or B4. We report a case of bilateral multifocal obstructive retinal vasculitis that developed after coxsackievirus A4 infection. A 60-year-old woman was referred to our department with bilateral visual disturbance that developed following flu-like symptoms. At the initial examination, best corrected visual acuity was 20/200 in the right eye and 20/50 in the left eye. The critical flicker frequency (CFF) was 23 Hz in the right eye and 27 Hz in the left eye. Fine white keratic precipitates with infiltrating cells were presented in the anterior chamber of both eyes, and multifocal retinal ischemic lesions were observed in the macula and posterior pole of both eyes. The retinal lesions corresponded with scotomas observed in Goldmann visual field test. On spectral domain-optical coherence tomography (SD-OCT), retinal lesions were depicted as hyper-reflective regions in the inner retina layers in both eyes, and disruption of ellipsoid line in the left eye., Fluorescein angiography exhibited findings indicative of multifocal obstructive retinal vasculitis. The patient had a history of current hypertension treated with oral therapy and glaucoma treated with latanoprost eye drops. Blood test for coxsackievirus antibody titers revealed that A4, A6, A9, B1, B2, B3, and B5 were positive (titers: 8-32). Abdominal skin biopsy of necrotic tissue suggested vascular damage caused by coxsackievirus. The general symptoms improved after 6 weeks, and the multifocal retinal ischemic lesions were partially resolved with residual slightly hard exudates. Only coxsackievirus A4

  20. 50 CFR 648.8 - Vessel identification.

    Science.gov (United States)

    2010-10-01

    ... 50 Wildlife and Fisheries 8 2010-10-01 2010-10-01 false Vessel identification. 648.8 Section 648.8... identification. (a) Vessel name and official number. Each fishing vessel subject to this part and over 25 ft (7.6... or ocean quahog vessels licensed under New Jersey law may use the appropriate vessel identification...