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Sample records for wegener granulomatosis

  1. Granulomatose de Wegener Wegener's granulomatosis

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    Telma Antunes

    2005-07-01

    Full Text Available A granulomatose de Wegener caracteriza-se por vasculite necrosante granulomatosa que acomete preferencialmente vias aéreas superiores, inferiores e rins. Seu diagnóstico é feito associando-se as manifestações clínicas, radiológicas (multiplos nódulos escavados e os achados anatomopatológicos e o anticorpo anticitoplasma de neutrófilos positivo. O tratamento com corticosteróides e ciclofosfamida leva a 90% de remissão da doença em 1 ano.Wegener's granulomatosis is characterized by granulomatous necrotizing vasculitis that primarily affects the airways (upper and lower and the kidneys. The diagnosis is made by analyzing the clinical and radiological manifestations (multiple pulmonary cavitations, together with the pathological findings and results of the test for cytoplasmic-pattern antineutrophil cytoplasmic antibodies. Treatment consists of corticosteroids and cyclophosphamide and leads to remission of the disease within one year in 90% of cases.

  2. Staphylococcus aureus and Wegener's granulomatosis

    NARCIS (Netherlands)

    Popa, ER; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2002-01-01

    Wegener's granulomatosis (WG) is a form of systemic vasculitis. It is characterized by granulomatous inflammation in the upper and lower airways, vasculitis and necrotizing glomerulonephritis, and is strongly associated with antineutrophil cytoplasmic antibodies against proteinase 3, Since the etiol

  3. Wegener's granulomatosis--an etiology of acute pancreatitis.

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    Joshipura, Vismit P; Haribhakti, Sanjiv P; Pandya, Sapan C; Soni, Harshad N; Patel, Nitin R

    2007-01-01

    Wegener's granulomatosis is a systemic disease that usually involves the upper respiratory tract and kidneys. We report a 47-year-old man with Wegener's granulomatosis that presented as acute pancreatitis.

  4. Pulmonary Localization Revealing Wegener's Granulomatosis

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    Mona Mlika

    2010-01-01

    Full Text Available Wegener's granulomatosis (WG is the most frequent antineutrophil cytoplasmic antibody (ANCA–associated vasculitis. It affects mainly the upper airways, lungs, and kidneys. Two forms are identified: systemic and limited. We describe three cases of limited WG diagnosed during a 7-year period. Our aim is to report three localized forms of WG and to put emphasis on the necessity of differentiating localized from systemic forms because of their different prognoses and manner of management. Our study contained two men and one woman with a mean age of 43 years. All our patients were symptomatic and presented with nonspecific respiratory signs. The cANCA were positive in all patients. The imaging findings consisted of cavitary masses. The diagnosis was based on surgical lung biopsy in all cases. All patients were put on cyclophosphamide and prednisolone. Only one patient presented with renal complications after a 2-year follow-up period. The two other patients did not present complications after, respectively, 1 month and 1 year of follow-up. These case reports put emphasis on a rare form of WG, the limited form. The low number of patients, due to the rarity of this disease, does not allow us to delineate the characteristics and the differences between this form and the systemic form, but we highlight the necessity of future investigations in order to explore the pathogenesis, therapeutic, and prognosis differences between these two subsets.

  5. Kidney involvement in a wegener granulomatosis case

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    Gioacchino Li Cavoli

    2012-01-01

    Full Text Available 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.

  6. Diagnostic Consideration for Sinonasal Wegener's Granulomatosis Clinically Mistaken for Carcinoma

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    La Rosa, Cristina; Emmanuele, Carmela; Tranchina, Maria Grazia; Ippolito, Massimo; Cosentino, Sebastiano; Saita, Vincenzo; Fraggetta, Filippo

    2013-01-01

    We report a case of Wegener's granulomatosis clinically mistaken for carcinoma in a 21-year-old girl presenting with an ulcerated mass of the nasopharynx associated with enlarged laterocervical nodes. The lesion was clinically suspected as malignant on the basis of clinical and radiological findings (namely, computed tomography scan and positron emission tomography). However, multiple biopsies were not conclusive for malignancy showing histological change suggestive of Wegener's granulomatosis. A serum determination of cANCA supported the diagnosis of Wegener's granulomatosis. Clinical findings and image studies suggested an erroneous diagnosis of malignancy whereas a definitive diagnosis of Wegener's granulomatosis was achieved only after repeated biopsies thus leading to a correct therapeutic approach. The Wegener granulomatosis must be added to the list of the differential diagnoses of the masses of the nasopharynx associated with or without enlarged laterocervical nodes. PMID:24106630

  7. Deoxyspergualin in relapsing and refractory Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Flossmann, O; Baslund, B; Bruchfeld, A

    2008-01-01

    at entry and prednisolone doses adjusted according to clinical status. Deoxyspergualin, 0.5 mg/kg per day, was self-administered by subcutaneous injection in six cycles of 21 days with a 7-day washout between cycles. Cycles were stopped early for white blood count less than 4000 cells/mm(3). The primary......-threatening (> or = grade 3) treatment-related adverse events occurred in 24 (53%) patients mostly due to leucopaenias. CONCLUSIONS: Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener's granulomatosis. Adverse events were common but rarely led...

  8. Massive intracerebral hemorrhage associated with Wegener granulomatosis.

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    Ceri, Mevlut; Ortabozkoyun, Levent; Unverdi, Selman; Kirac, Mustafa; Duranay, Murat

    2012-06-01

    Wegener granulomatosis (WG) is a necrotizing granulomatous vasculitis that predominantly affects airways and kidneys. But central nervous system involvement (7-11%) is an uncommon. Massive ICH may occur in the course of WG, and this serious condition is related with high risk of mortality. Therefore, the new treatment strategies may be considered in addition to classical practices in serious organ involvement and recurrent attack. Here, we present an adult patient with WG whose disease was complicated by a massive intracerebral hemorrhage (ICH), which subsequently led to death.

  9. Linfoma de Burkitt en un portador de granulomatosis de Wegener

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    Kryssia Rodríguez-Castro

    2005-10-01

    Full Text Available Se reporta el caso de un paciente costarricense portador de granulomatosis de Wegener, en tratamiento con ciclofosfamida y prednisona, quien desarrolla un linfoma de Burkitt que lo lleva a la muerte. Se menciona la posible relación entre estas dos patologías.This is a report of a costarrican male with Wegener' s granulomatosis undergoing treatment with prednisone and cyclophosphamide, he developed a fatal Burkitt' s lymphoma. The possible relationship between these two disorders is addressed.

  10. Bronchoplastic procedure for an unusual indication--Wegener's granulomatosis.

    LENUS (Irish Health Repository)

    Soo, Alan

    2009-09-01

    Wegener\\'s granulomatosis (WG) is a systemic vasculitic condition that commonly affects the lung and kidneys. With improvement in medical therapy, airway complications are increasingly encountered and are difficult to manage. Here, we present a case whereby a patient presenting with airway complication is successfully treated with surgery.

  11. Wegener's granulomatosis simulated by a T cell lymphoma of the lung

    OpenAIRE

    1991-01-01

    A case of primary T cell lymphoma of the lung associated with antineutrophil cytoplasmic antibody simulated Wegener's granulomatosis, the patient having features compatible with but not diagnostic of Wegener's granulomatosis.

  12. Wegener's granulomatosis in childhood

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    McHugh, K.; Manson, D. (Hospital for Sick Children, Toronto, ON (Canada). Dept. of Diagnostic Imaging); Eberhard, B.A.; Shore, A.; Laxer, R.M. (Hospital for Sick Children, Toronto, ON (Canada). Div. of Rheumatology)

    1991-12-01

    The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients - one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG. (orig.).

  13. Sinonasal Wegener's granulomatosis: CT characteristics

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    Benoudiba, F.; Marsot-Dupuch, K.; Rabia, Hadj M.; Lasjaunias, P. [Neuroradiology Department, Bicetre Hospital, 78 rue du General Leclerc, 94275 Le Kremlin Bicetre (France); Cabanne, J. [Department of Internal Medecine, Saint-Antoine Hospital, 184 rue du Faubourg Saint-Antoine, 75012 Paris (France); Bobin, S. [Head and Neck Department, Bicetre Hospital, 78, rue du General Leclerc, 94275 Le Kremlin Bicetre (France)

    2003-02-01

    Wegener's granulomatosis (WG) is a severe and potentially lethal granulomatosis. Even though no specific radiological criteria exist, CT may suggest the correct diagnosis at an early stage. Recent improvement in the prognosis is related to earlier diagnosis, allowing the initiation of efficient and specific treatment before any severe complications occur. We reviewed a series of WG cases in order to establish the CT diagnostic criteria. (orig.)

  14. Wegener's granulomatosis : description of a case with oral manifestation

    OpenAIRE

    Reboll Ferrer, Rosa María; Zapater Latorre, Enrique; Calabuig Crespo , Consuelo; Basterra Alegría, Jorge

    2010-01-01

    Wegener?s granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolutio...

  15. Pancreatic mass as an initial presentation of severe Wegener's granulomatosis

    Science.gov (United States)

    Valerieva, Yana; Golemanov, Branimir; Tzolova, Nadezhda; Mitova, Rumiana

    2013-01-01

    Acute pancreatitis or a pancreatic mass is a very rare initial presentation of Wegener's granu-lomatosis. A 62-year-old woman presented with tumor-like pancreatitis and otitis media Abdominal ultrasound and magnetic resonance suggested the presence of pancreatic tumor. Ultrasound-guided fine needle aspiration was negative. Distal pancreatic resection and splenectomy were performed and histopathology proved Wegener's vasculitis of the pancreas and spleen. Azathioprine and steroids were subsequently started and six months later the patient was asymptomatic. Involvement of the pancreas could be considered as a presenting symptom of Wegener's vasculitis. PMID:24714250

  16. [The first clinical description of granulomatosis with polyangiitis (known before as Wegener's granulomatosis)].

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    Mercado, Ulises

    2017-01-01

    Before 1950, cases of necrotizing vasculitis were commonly published in journals of pathology. Most of these cases were designated as polyarteritis nodosa. In 1952, the pathologist Pearl Zeek critically reviewed and summarized the literature dealing with polyarteritis nodosa and first grouped the different types of necrotizing vasculitis. But she omitted some types of not well-characterized vasculitis, among them granulomatosis with polyangiitis (Wegener's granulomatosis).

  17. Incidence of granulomatosis with polyangiitis (Wegener`s) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence ...

  18. Incidence of granulomatosis with polyangiitis (Wegener`s) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence ...

  19. Wegener's granulomatosis: description of a case with oral manifestation.

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    Reboll-Ferrer, Rosa Maria; Zapater-Latorre, Enrique; Calabuig-Crespo, Consuelo; Basterra-Alegría, Jorge

    2010-07-01

    Wegener's granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener's granulomatosis with oral manifestation, which began as chronic mastitis.

  20. An atypical case of Wegener`s granulomatosis complicated by sepsis and coxitis; Ziarniniakowatosc Wegenera o nietypowej lokalizacji powiklana posocznica i ropnym zapaleniem stawu biodrowego

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    Makowski, A.; Faflik, J. [Centrum Zdrowia Matki Polki, Lodz (Poland)

    1993-12-31

    There is presented an atypical case Wegener`s granulomatosis of maxillo-nasal region without bony destruction. The case is unusual because of sepsis and purulent coxitis. The patient responded well to treatment with vincristine and cyclophosphamide. ANCA (anti-neutrophil cytoplasmic antibodies) assays have very good sensitivity and specificity for Wegener`s granulomatosis. (author). 17 refs, 2 figs.

  1. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sørensen, Inge Juul; Mellemkjaer, Lene

    2008-01-01

    To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG.......To describe the incidence of malignancies in a cohort of Danish patients with Wegener's granulomatosis (WG) and to investigate the cancer risk associated with cyclophosphamide (CYC) -therapy in WG....

  2. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis.

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    Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur

    2003-03-01

    The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.

  3. Meningeal involvement in Wegener's granulomatosis is associated with localized disease.

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    Di Comite, G; Bozzolo, E P; Praderio, L; Tresoldi, M; Sabbadini, M G

    2006-01-01

    Meningeal involvement is a rare occurrence in Wegener's Granulomatosis (WG). A Medline search uncovered only 48 previously reported cases. Here we describe the clinical features of meningeal involvement in WG and to evaluate the association with systemic disease extension. Through a systematic literature review of papers concerning meningeal involvement in WG, we collected and analysed data about sex, age, disease extension, symptoms, cerebrospinal fluid examination, imaging, ANCA and histology about previously reported patients. Headache is almost always the first symptom of meningeal involvement in WG. Later in the course of the disease other abnormalities may develop. Among them cranial nerve palsy, seizures and encephalopathy are the most frequent. Diagnosis is obtained by neuroimaging, which may disclose two distinct patterns of meningeal thickening: diffuse or focal. 62.9% of patients tests positive for ANCA. Histology typically shows necrotizing granulomatosis. Meningeal involvement is by far more frequent in the setting of localized WG. Meningitis is a rare complication of WG. It usually develops in patients with localized disease who are more likely to have destructive lesions of the upper airways. It may be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis, with little or no vasculitis.

  4. Acute Pancreatitis as the First Presentation of Wegener's Granulomatosis

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    Mohammed Abu-Hilal

    2008-05-01

    Full Text Available Context Wegener’s granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. An association between acute pancreatitis and Wegener's granulomatosis is rarely reported and is even rarer as the first presentation. This can result in diagnostic difficulty and may allow severe pancreatitis to develop with potentially poor outcome. Case report We report a rare case with fatal outcome of vasculitis consistent with Wegener’s granulomatosis presenting as acute pancreatitis in a 20-year-old female. The patient was admitted with worsening abdominal pain associated with nausea and loss of appetite. Accepted causes of acute pancreatitis were excluded and granulomatous vasculitis of the pancreas was confirmed from immunological profile, computed tomography and histology. As the disease progressed the patient experienced cutaneous, pulmonary, renal and severe gut involvement. Thirteen months from diagnosis the patient died of multi-organ failure despite appropriate surgical and immunosuppressive therapy. Conclusion Vasculitic disease of the pancreas is rare but should be considered when other causes have been appropriately ruled out. Careful radiological, immunological and histological diagnosis is necessary and early immunosuppressant therapy in conjunction with advice from immunologists is essential to avoid the poor outcome reported in this and other case reports.

  5. Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature

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    Lohrmann, Christian; Uhl, Markus; Kotter, Elmar; Burger, Dieter; Ghanem, Nadir; Langer, Mathias

    2005-03-01

    Wegener granulomatosis is a multisystem disease of unknown cause characterized by a necrotizing granulomatous vasculitis. In comparison to other vasculitides, the lung is the most common organ involved in wegener granulomatosis presenting with a very aggressive airways pathology and chronic relapsing course. Chest radiographs fail to describe the pattern and distribution of thoracic pathology sufficiently, and CT has shown to be more sensitive for detecting lung involvement. We present the CT findings of 57 patients with wegener granulomatosis and a review of the literature.

  6. Cardiopatía valvular en un paciente con granulomatosis de Wegener Valvular cardiopathy in a patient with Wegener's granulomatosis

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    Luis E Silva

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.Wegener's granulomatosis is an autoimmune necrotizing vasculitis with a reported prevalence of 3/100.000 inhabitants. It is a multisystemic disease, involving mainly the upper and lower respiratory tract, the central nervous system and the kidneys. The frequency of cardiac involvement varies depending on the series studied, but oscillates around 6%; however, the clinical manifestations are rare. The valvular involvement is reported as aortic or mitral insufficiency secondary to infiltration of the leaflets, or as aortic root dilatation. Treatment is based on the control of the disease, the management of heart failure and surgical intervention of the involved valve, as directed.

  7. Diabetes Insipidus as a Complication of Wegener's Granulomatosis and Its Treatment with Biologic Agents

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    Joanna Rosalind Cunnington

    2009-01-01

    Full Text Available Wegener's granulomatosis of the pituitary gland resulting in diabetes insipidus is a rare complication of the disease. Standard treatment for Wegener's granulomatosis involves a combination of prednisolone and cylophosphamide, however biologic agents are now being used in refractory cases. We report three cases of patients with diabetes insipidus as a complication of Wegener's granulomatosis who were treated with biologic agents. All three cases showed clinical response to treatment with biologic agents including rituximab and alemtuzumab and two cases demonstrated improvement in pituitary gland abnormalities by MRI. Clinicians should be aware that diabetes insipidus can present as a complication of Wegener's granulomatosis and that biologic therapies may be effective in refractory cases.

  8. A disease activity score for ENT involvement in granulomatosis with polyangiitis (Wegener's)

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    Del Pero, Marcos Martinez; Chaudhry, Afzal; Rasmussen, Niels

    2013-01-01

    Accurate assessment of disease activity in patients with otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (ENT/GPA) is necessary for treatment decisions and clinical trials. We have designed a disease activity score (ENT/GPA DAS) for this purpose....

  9. Wegener's granulomatosis patients show an adequate antibody response to influenza vaccination

    NARCIS (Netherlands)

    Holvast, A.; Stegeman, C. A.; Benne, C. A.; Huckriede, A.; Wilschut, J. C.; Palache, A. M.; Kallenberg, C. G. M.; Bij, M.

    2009-01-01

    Objectives: Wegener's granulomatosis (WG) is a systemic vasculitis characterised by relapsing and remitting disease activity. Immunosuppressive drugs are used to control disease, but increase susceptibility to infection. Therefore, influenza vaccination should be considered in WG patients. This stud

  10. Impaired health-related quality of life in patients treated for Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Sigaard, Lene; Bjørner, Jakob Bue;

    2010-01-01

    To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL.......To investigate whether patients with Wegener's granulomatosis (WG) experience reduced health-related quality of life (HRQOL) after accomplishment of remission, and to study the influence of WG-associated organ damage on HRQOL....

  11. Wegener's granulomatosis: chest computed tomography findings; Granulomatose de Wegener: aspectos na tomografia computadorizada de torax

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    Monteiro, Evelise de Azevedo; Marchiori, Edson; Martins, Erick Malheiro Leoncio [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Hospital de Base; Cerqueira, Elza Maria F.P. de [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia; Irion, Klaus L. [Pavilhao Pereira Filho, Porto Alegre, RS (Brazil); Araujo Neto, Cesar de [Bahia Univ., Salvador, BA (Brazil). Dept. de Radiologia; Souza, Rodrigo Azeredo de [Universidade Federal, Rio de Janeiro, RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia

    2003-03-01

    Wegeners granulomatosis is an inflammatory systemic necrotizing idiopathic vasculitis affecting mainly small vessels. In this paper we analyzed the computed tomography findings of seven patients with Wegeners granulomatosis. The most common findings were masses and nodules (71.4%), areas of ground glass attenuation (57.1%), consolidation (42.8%), halo sign (42.8%), cavitation (42.8%), interlobular septa thickening (14.2%), bronchial wall thickening (14.2%) and pleural effusion (14.2%). Although radiological findings are mostly nonspecific, computed tomography and particularly high-resolution computed tomography are important tools in the evaluation of disease activity when evaluated in association with bronchoscopy findings and clinical data. (author)

  12. Massive Alveolar Hemorrhage During Wegener Granulomatosis: a Case Report

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    Gökhan Perincek

    2011-11-01

    Full Text Available This is a presentation of Wegener Granulomatosis (WG disease. Even though the lungs are rarely affected. massive alveolar hemorrhage is seen which leads to mortality. The patient was a 28 year old man. His illness was diagnosed as WG and glomerulonephritis a year previously and he was treated by administration of methylprednisolone orally. He had been treated irregularly. He applied to the emergency service with hemoptysis and asthma complaints two days earlier. After the results of his examination Hb: 3.6 gr/dl, Htc:10.3%, Üre:131 mg /dl, kreatini: 7.7 mg/dl, pH: 7.41, pO2: 55 mmHg, pCO2:33 mmHg, and being diagnosed as alveolar consolidation on lung X-ray, he was taken to the intensive care unit with a diagnosis of a massive alveolar hemorrhagei. He was intubated and attached to mechanical ventilation. He was treated with parenteral 1 mg/kg/day methylprednisolone and, siklofosfamid 2 mg/kg/day. He was extubated on the 21st day. He was taken to the chest service department on 24th day. He is still being treated.

  13. Renal cancer and Wegener's granulomatosis: a case report

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    Bumbasirevic Uros

    2011-12-01

    Full Text Available Abstract Wegener's granulomatosis (WG is a systemic disorder characterized by necrotizing vasculitis involving the respiratory tract, and in most cases, the kidneys. The most common manifestation of WG in the kidneys is segmental necrotizing glomerulonephritis. The presence of a renal mass as a manifestation of WG is rare. We report a patient with WG in whom a CT scan revealed an infiltrating mass in the lower portion of the left kidney. After surgical exploration, we performed an open radical nephrectomy. Histopathology showed clear cell type renal cell carcinoma (RCC. RCC associated with WG has been reported in only a few cases, and in most of them, the diseases started simultaneously, suggesting common pathogenetic pathways. Long-term immunosuppressive treatment is a known risk factor in the development of malignancies, so occurrence of RCC in WG has been proposed as a side effect of cyclophosphamide treatment. Furthermore, it is important to make a differential diagnosis between RCC and pseudotumors in WG as they cannot be distinguished solely on basis of imaging findings. Due to the higher risk of urologic malignancies, more frequent checkups and screening of WG patients should be considered.

  14. Kaposi's sarcoma in an elderly man with Wegener's granulomatosis treated with cyclophosphamide and corticosteroids.

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    Erban, S B; Sokas, R K

    1988-05-01

    The association of Kaposi's sarcoma with malignant lymphoreticular diseases and immunosuppressive therapy is well documented. This report describes an elderly man who presented with fulminant Wegener's granulomatosis that responded to treatment with cyclophosphamide and corticosteroids. Rapidly progressing cutaneous Kaposi's sarcoma developed ten weeks after the start of immunosuppressive therapy yet regressed on discontinuation of the corticosteroid therapy, despite continuation of cyclophosphamide therapy. To our knowledge, this is the first reported case of Kaposi's sarcoma occurring in association with Wegener's granulomatosis. The literature on Kaposi's sarcoma in immunosuppressed patients is reviewed.

  15. Persistent expansion of CD4(+) effector memory T cells in Wegener's granulomatosis

    NARCIS (Netherlands)

    Abdulahad, W. H.; van der Geld, Y. M.; Stegeman, C. A.; Kallenberg, C. G. M.

    2006-01-01

    In order to test the hypothesis that Wegener's granulomatosis (WG) is associated with an ongoing immune effector response, even in remission, we examined the distribution of peripheral naive and memory T-lymphocytes in this disease, and analyzed the function-related phenotypes of the memory T-cell p

  16. Plasma levels of soluble endothelial cell protein C receptor in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Boomsma, MM; Stearns-Kurosawa, DJ; Stegeman, CA; Raschi, E; Meroni, PL; Kurosawa, S; Tervaert, JWC

    Elevated soluble thrombomodulin (sTM) levels are an accepted marker of endothelial damage. The physiological significance of plasma endothelial protein C receptor (sEPCR) levels is not known. To assess the relevance of this plasma protein in Wegener's granulomatosis (WG), sEPCR levels were measured

  17. Wegener´s granulomatosis in a young patient preceded by localized cutaneous manifestations

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    Jesper Smit

    2011-10-01

    Full Text Available Wegener´s granulomatosis (WG is a rare, systemic vasculitis involving multiple organs. The clinical presentation is highly diverse, and there is considerable risk of mortality if diagnosis and treatment are delayed. We present a case illustrating that patients with WG may initially present with localized cutaneous symptoms and signs.

  18. Cranial nerve palsy in Wegener's granulomatosis--lessons from clinical cases

    DEFF Research Database (Denmark)

    Nowack, Rainer; Wachtler, Paul; Kunz, Jürgen;

    2009-01-01

    The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging...

  19. Dilatation tracheoscopy for laryngeal and tracheal stenosis in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Schokkenbroek, Ada A.; Franssen, Casper F. M.; Dikkers, Frederik G.

    2008-01-01

    Wegener's granulomatosis (WG) frequently involves the subglottis and trachea and may compromise the upper airway. The objective of this study is to evaluate retrospectively the effect of treatment of subglottic stenosis (SGS) and tracheal stenosis (TS) by dilatation tracheoscopy (DT) in patients wit

  20. Enhanced endothelium-dependent microvascular responses in patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Nienhuis, Hans L.A.; De Leeuw, Karina; Smit, Andries J.; Bijzet, Johan; Stegeman, Coen A.; Kallenberg, Cees G.M.; Bijl, Marc

    2007-01-01

    Objective. To assess endothelial cell (EC) function of the cutaneous microcirculation in patients with Wegener's granulomatosis (WG) and to relate EC function to EC activation and presence of atherosclerosis. Methods. We studied 28 WG patients with inactive disease and 28 age and sex matched control

  1. Wegener's granulomatosis with unusual cavernous sinus and sella turcica extension

    Energy Technology Data Exchange (ETDEWEB)

    Hermann, M.; Bobek-Billewicz, B. [Dept. of Radiology, Medical University of Gdansk (Poland); Bullo, B.; Hermann, A.; Rutkowski, B. [Dept. of Nephrology, Medical University of Gdansk (Poland)

    1999-07-01

    Intracerebral extension of Wegener's granulomatosis (WG) is rare. We present a patient with oculomotor and trochlear nerve palsy with histologically proved WG. An MR examination revealed granulomatous tissue in nasal cavity, paranasal sinuses with meningeal infiltration, and uncommon penetration into cavernous sinus and sella turcica. The MR images before and during pharmacological therapy are presented. (orig.)

  2. Detection of immune deposits in skin lesions of patients with Wegener's granulomatosis

    NARCIS (Netherlands)

    Brons, RH; de Jong, MCJM; de Boer, NK; Stegeman, CA; Kallenberg, CGM; Tervaert, JWC

    2001-01-01

    Background-Wegener's granulomatosis (WG) is considered a pauci-immune systemic vasculitis based on the absence of immune deposits in renal biopsies of patients with active disease. In animal models of antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis, immune deposits along the

  3. Case of granulomatosis with polyangiitis (Wegener's granulomatosis) manifested with asymptomatic intracerebral hemorrhage.

    Science.gov (United States)

    Takaoka, Hirokazu; Hashimoto, Atsushi; Nogi, Shinichi; Iwata, Kanako; Futami, Hidekazu; Arinuma, Yoshiyuki; Shimada, Kota; Nakayama, Hisanori; Komiya, Akiko; Furukawa, Hiroshi; Matsui, Toshihiro; Tohma, Shigeto

    2013-01-01

    A 46-year-old man, who had had sinusitis, developed bilateral omalgia, petechiae on his lower extremities and a congested right eye. A blood test detected elevated serum C-reactive protein level. Computed tomography incidentally found an acute lesion of thalamic hemorrhage without neurological symptoms and no specific therapy was given at the time. Thereafter, he developed vertigo, vomiting and pneumonia for which antibiotics were ineffective. He was referred and admitted to our hospital. Further, aural and renal lesions, and presence of serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) confirmed his diagnosis of granulomatosis with polyangiitis (Wegener's) (GPA). With corticosteroid and cyclophosphamide therapy, his symptoms disappeared in two months along with faded PR3-ANCA. Afterward he showed neither new cerebral lesion nor symptom. This is a rare case of GPA manifested with asymptomatic intracerebral hemorrhage. It should be noted that GPA could cause various manifestations in central nervous system such as a fatal or an asymptomatic hemorrhagic lesion, which might respond to immunosuppressive therapy.

  4. Limited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report.

    Science.gov (United States)

    Pai, Sushma; Panda, Mukta

    2008-12-23

    Rheumatoid arthritis has varied pleuroparenchymal manifestations. Wegener's granulomatosis can develop in an established case of rheumatoid arthritis and this association although previously reported is very rare. A 60-year-old lady had been diagnosed with rheumatoid arthritis on the basis of her clinical symptoms and serological tests which were positive RA factor and anti-CCP antibodies. Her rheumatoid arthritis activity had been mild and well controlled with hydroxychloroquine and low dose prednisone. She presented with a productive cough and right-sided pleuritic chest pain. CT scan of the chest showed three lung nodules with increased uptake on PET CT scan, raising concerns for an inflammatory or malignant process. The differential diagnosis included rheumatoid nodules, infections or malignancy. A CT-guided needle biopsy of the largest nodule was undertaken which showed vasculitis typical of Wegener's granulomatosis. Stains and cultures of the biopsy specimen were negative for bacteria, fungi and acid fast bacilli. A panel of serological tests for vasculitis were checked and showed elevated titers of cANCA and anti-proteinase 3 antibodies. Urine analysis and CT scan of paranasal sinuses was normal. Since the upper respiratory tract and the kidneys were spared a diagnosis of limited Wegener's granulomatosis affecting only the lungs was made. Due to the toxicity of cyclophosphamide, her relatively mild disease sparing the kidneys and the underlying rheumatoid arthritis, weekly methotrexate was started and low dose prednisone was continued. She had marked symptomatic improvement and complete resolution of the nodules was documented on subsequent imaging. Wegener's granulomatosis developing in a patient with rheumatoid arthritis is very rare but should be considered as it warrants a different and possibly more aggressive treatment approach.

  5. Incidence of granulomatosis with polyangiitis (Wegener's) in Greenland and the Faroe Islands

    DEFF Research Database (Denmark)

    Faurschou, M; Helleberg, M; Obel, N

    2013-01-01

    Previous studies suggest that the incidence of granulomatosis with polyangiitis (Wegener's; GPA) increases along a south-north gradient in the Northern Hemisphere with an incidence of 8.0/million/year reported for the population of Northern Norway. In the present study, we assessed the incidence...... of GPA in the predominantly Inuit population of Greenland and in the Caucasian population of the Faroe Islands....

  6. Testicular Involvement in Wegener Granulomatosis: Case Report and Review of the literature

    Directory of Open Access Journals (Sweden)

    Mehmet Nuri TURAN

    2012-05-01

    Full Text Available Wegener granulomatosis (WG is a type of necrotising vasculitis that affects mainly the respiratory tract, paranasal sinuses and the kidneys. Urogenital involvement is rare; mainly affecting the prostate gland. Testicular involvement in WG is exceptional. We report a 50-year-old male patient with hematuria, proteinuria and testicular pain and renal failure diagnosed as WG by testicular and kidney biopsy. We also provide a review of the literature.

  7. Granulomatose de Wegener e estenose subglótica: descrição de caso Wegener's granulomatosis and subglottic stenosis: case description

    Directory of Open Access Journals (Sweden)

    Fernanda Guidolin

    2004-06-01

    Full Text Available Descreve-se o caso de um paciente com Granulomatose de Wegener, em tratamento com esteróides e ciclofosfamida, que, apesar do tratamento, evoluiu com dispnéia progressiva e rouquidão. A investigação mostrou tratar-se de uma estenose subglótica, sendo a dispnéia prontamente aliviada com a traqueostomia. São discutidos diagnósticos diferenciais e modalidades de tratamentos de estenose subglótica em portadores de Wegener.We describe a patient with Wegener Granulomatosis being treated with steroids and cyclofosfamide that developed progressive dyspnea and hoarseness despite of treatment. Investigation showed a subglotic stenosis. The dyspnea was relieved by tracheotomy. We discuss the differential diagnosis as well as a number of treatment modalities in patients with Wegener granulomatosis and subglottic stenosis.

  8. Polymicrobial Infections in the Early Period of Kidney Transplantation in a Case with Wegener's Granulomatosis

    Directory of Open Access Journals (Sweden)

    Funda COŞKUN

    2011-01-01

    Full Text Available Wegener's granulomatosis (WG is a disorder that causes necrotizing granulomatosis vasculitis particularly of the upper respiratory tract, lung and kidney. A 43-year-old male who had been treated with hemodialysis because of renal insufficiency due to WG underwent live donor renal transplantation. Pulmonary infiltrates were detected on the postoperative 4th day and antibiotic therapy was started with a diagnosis of sepsis and pulmonary infection. Dialysis treatment was also started due to the degradation of renal function for the patient who was intubated during follow-up. Non-invasive mechanical ventilation (BiPAP treatment was started after extubation. The graft and respiratory function improved during clinical follow-up. Resistant hospital infections, causing respiratory failure and systemic complications, were facilitated by a long history of hospitalization before transplantation, the presence of WG and immunosupression and were successfully treated with a multidisciplinary approach.a

  9. Wegener`s granulomatosis and mucoromycosis: A case study and review of literature

    Directory of Open Access Journals (Sweden)

    Sayyed Mojtaba Abtahi

    2012-01-01

    Full Text Available Mucormycosis is a fatal invasive infection which mostly involves diabetic or immunosuppressed patients. Early diagnosis, improving immunosuppression, systemic antifungal therapy, and surgical debridement are necessary for successful treatment. In this case study, we represent a known case of Wegener′s granulomatosis (WG, with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline.

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

    DEFF Research Database (Denmark)

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

    2011-01-01

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

  11. Pituitary involvement in Wegener ' s granulomatosis: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    陶建瓴; 董怡

    2003-01-01

    Wegener ' s Granulomatosis (WG) is a multi-system disorder characterized by necrotizing granulomas in the upper and/or lower respiratory tract, with or without focal necrotizing glomerulonephritis and other systemic vasculitis.1 Clinical manifestations may vary with the site of involvement and the extension of the lesions. Pituitary involvement is rare, and to our knowledge no more than ten cases with this complication have been published in the literature in English. We report here an additional WG case complicated by pan-hypopituitarism.

  12. Increased morbidity from ischemic heart disease in patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sørensen, Inge Juul;

    2009-01-01

    OBJECTIVE: Experimental studies indicate that patients with Wegener's granulomatosis (WG) experience accelerated atherosclerosis. The purpose of this study was to investigate whether the occurrence of overt ischemic heart disease (IHD) is increased in WG. METHODS: A total of 293 WG patients were...... MI were found in patients who were > or =50.0 years of age at the time of diagnosis of WG, in male patients, and in patients who received high cumulative doses of cyclophosphamide. CONCLUSION: Compared with the background population, WG patients seem to experience an increased number of both early...

  13. Lingual infarction in Wegener's Granulomatosis: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Brizman Eitan

    2008-08-01

    Full Text Available Abstract Wegener's granulomatosis (WG is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding oral manifestations of WG and present a case of lingual infarction, an extremely rare oral lesion associated with WG, in a severe, rapidly progressive and ultimately fatal form of the disease.

  14. Granulomatous renal pseudotumor in Wegener's granulomatosis: imaging findings in one case

    Energy Technology Data Exchange (ETDEWEB)

    Verswijvel, G.; Eerens, I.; Oyen, R. [Department of Radiology, University Hospitals. Catholic University of Leuven, Leuven (Belgium); Messiaen, T. [Department of Nephrology, University Hospitals. Catholic University of Leuven, Leuven (Belgium)

    2000-08-01

    Wegener's granulomatosis is a clinico-pathological entity characterized by necrotizing granulomatous angiitis involving the upper- and/or lower respiratory tract and the kidneys. Renal involvement is usually characterized by a rapidly progressive necrotizing glomerulonephritis. A case is presented of a patient who developed renal failure and presented a solitary pseudotumoral lesion in the upper pole of the left kidney. Imaging characteristics on US, CT and MR imaging are discussed. The diagnosis was confirmed with ultrasound-guided needle biopsy. (orig.)

  15. [Systemic diseases--the significance of early diagnosis exemplified by systemic lupus erythematosus and Wegener's granulomatosis].

    Science.gov (United States)

    Türk, H

    1993-12-01

    New therapeutic modalities have shown remarkable advances in the fields of systemic lupus erythematosus and Wegener's granulomatosis. For an optimal clinical outcome therapy has to be started early and must be adapted to disease activity. Concerning these two points early diagnosis is essential. This aim can be reached by a detailed evaluation of the patient's history by focusing on early symptoms and on typical clinical constellations, taking into account that both diseases show a great variability and sometimes even an insidious course. Immunological tests, capillary microscopy, echocardiography and computed tomography can be very helpful in the diagnosis of these diseases.

  16. Prevalence of hearing impairment in patients with rheumatoid arthritis, granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or systemic lupus erythematosus.

    Science.gov (United States)

    Rahne, Torsten; Clauß, Franziska; Plontke, Stefan K; Keyßer, Gernot

    2017-07-01

    Hearing loss in patients with autoimmune diseases, such as systemic lupus erythematosus (SLE), granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), or rheumatoid arthritis (RA), is controversial. Many studies lack measurements of bone-conduction thresholds to sufficiently differentiate between sensorineural hearing loss and conductive hearing loss. In addition, many studies lack control groups or comparisons to an age-related normal hearing threshold. This study investigates hearing performance with an extended audiological battery using psychoacoustic and objective measures. A total of 22 adults with RA, 16 with GPA, 20 with SLE, and two age- and gender-matched control groups (n = 34 for GPA and RA and n = 42 for SLE) were included. Pure-tone hearing thresholds, speech perception in quiet and noise, tympanometry, and high-resolution otoacoustic emissions were assessed. GPA patients exhibited impaired pure-tone hearing compared to the control group, whereas SLE and RA patients did not. In GPA patients, a larger air-bone gap indicated conductive hearing loss. In addition, speech perception was reduced exclusively in GPA patients. A significant correlation was found between hearing loss and both the cumulative steroid dose and number of organ manifestations in GPA and SLE patients. Our data indicate that GPA and SLE patients are at moderate-to-high risk of conductive hearing loss. In contrast, RA patients are at low risk of disease-associated hearing loss.

  17. Pseudotumor pulmonar como apresentação inicial de granulomatose de Wegener Lung pseudotumor as the initial presentation of Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Marcelo Fouad Rabahi

    2009-04-01

    Full Text Available A granulomatose de Wegener é uma doença inflamatória multissistêmica de etiologia desconhecida, que se caracteriza por vasculite de pequeno e médio calibre. As manifestações clínicas mais comuns envolvem o trato respiratório superior, pulmões e rins e as alterações encontradas nos exames de imagem são geralmente opacidades pulmonares ou nódulos múltiplos, bilaterais e, em 50% dos casos, cavitações. O tratamento é feito com corticoides e imunossupressores. Descreve-se um caso atípico de um homem de 61 anos cuja investigação inicial de um tumor pulmonar unilateral evidenciou, na verdade, Granulomatose de Wegener.Wegener's granulomatosis is a multisystemic inflammatory illness of unknown etiology, characterized by vasculitis of small and medium caliber vessels. The most common clinical manifestations involve the upper respiratory tract, lungs and kidneys. Common alterations in imaging studies include pulmonary opacities and bilateral multiple nodules, cavitations occurring in 50% of the cases. Treatment includes corticosteroids and immunosuppressants. We describe an atypical case of a 61-year-old man initially investigated due to suspicion of a unilateral lung tumor, which proved to be, in fact, a case of Wegener's granulomatosis.

  18. 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.

  19. Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis.

    Science.gov (United States)

    Spísek, Radek; Kolouchová, Elena; Jensovský, Jirí; Rusina, Robert; Fendrych, Pavel; Plas, Jaroslav; Bartůnková, Jirina

    2006-09-01

    Wegener granulomatosis (WG) is a systemic vasculitis of small and medium vessels. It predominantly affects the upper and/or lower respiratory airway and kidneys. Its pathogenesis is not fully understood. WG relatively frequently affects the nervous system (in 30-50% according to the different studies). Most frequently, it manifests as necrotizing vasculitis that leads to the peripheral neuropathies or to the cranial nerves palsy. Impairment of the central nervous system (CNS) is less frequent and occurs in 2-8% of patients. Three major pathogenetic mechanisms were described: CNS vasculitis, spreading of granulomas from the adjacent anatomical areas (paranasal cavities, orbit etc.), and new formation of granulomas in brain tissue. This case report describes patients in whom WG manifested in the form of localized skin involvement and combined CNS involvement that included pituitary gland. Atypical presentation of WG impedes and slows down the process of diagnosis and emphasizes the need for collaboration between medical specialists.

  20. Cardiopatía valvular en un paciente con granulomatosis de Wegener

    Directory of Open Access Journals (Sweden)

    Luis E. Silva, MD

    2012-01-01

    Full Text Available La granulomatosis de Wegener es una vasculitis necrotizante autoinmune cuya prevalencia reportada es de 3/100.000 habitantes. Tiene compromiso multisistémico, principalmente el tracto respiratorio superior e inferior, y el sistema nervioso central y renal. La frecuencia del compromiso cardiaco varía según las series estudiadas, pero oscila alrededor de 6%; sin embargo, las manifestaciones clínicas son poco frecuentes. El compromiso valvular se reporta como insuficiencia aórtica o mitral, secundaria a infiltración de las valvas o dilatación de la raíz aórtica. El tratamiento se basa en el control de la enfermedad, el manejo de la falla cardiaca y la intervención quirúrgica de la válvula comprometida según la indicación.

  1. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyangiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  2. [Tracheal resection for post-intubation subglottic stenosis in a patient with granulomatosis with polyanaiitis (Wegener)].

    Science.gov (United States)

    Stoica, Radu; Negru, Irina; Matache, Radu; MirunaTodor

    2014-01-01

    Granulomatosis with polyangiitis (GPA or Wegener) is a systemic autoimmune disease with inflammation of small- and medium-size vessels. It can affect practically any organ or system, but renal, respiratory andjoint systems are most frequently damaged. Positive pANCA antibodies can raise the suspicion of diagnosis. Subglottic stenosis is relatively frequent, in a quarter of patients, especially in the third decade women. The case presented is of an 80-year-old woman, recently diagnosed with pulmonary, renal and systemic manifestations of GPA and with a subglottic stenosis rapidly evolving towards endotracheal intubation, tracheostomy with mechanical ventilation and renal failure. Further evolution has been favorable under corticoid therapy. After weaning from the mechanical ventilation and30 days after the suppression of the tracheostomy, the patient developed a tracheal stenosis with mixed etiology, secondary to vasculitis and prolonged intubation with tracheostomy. Tracheal resection with termino-terminal anastomosis was performed in emergency with simple post-operative evolution and without late complications.

  3. Airway Surgery in Tracheostomised Patients with Wegener Granulomatosis Leading to Subglottic Stenosis.

    Science.gov (United States)

    Altun, Demet; Sivrikoz, Nükhet; Çamcı, Emre

    2015-10-01

    Wegener granulomatosis (WG) is a multisystemic disorder characterised by granulomatous inflammation of the respiratory system. The growing of proliferative tissue towards the larynx and trachea may cause airway obstruction on account of subglottic stenosis. In this situation, the surgical goal is to eliminate the airway obstruction by providing natural airway anatomy. While mild lesions do not require surgical intervention, in fixed lesions, surgical intervention is required, such as tracheostomy, laser resection and dilatation. In tracheostomised patients, granuloma formation surrounding the tracheostomy cannula may occur in the trachea. Inflammation and newly formed granulation tissue result in severe stenosis in the airways. During surgical treatment of such patients, airway management is important. In this case report, we will discuss gas exchange and airway management with jet ventilation (JV) during excision of the granulation tissue with endolaryngeal laser surgery, leading to subglottic stenosis in tracheostomised patients in WG.

  4. Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

    Directory of Open Access Journals (Sweden)

    Gustavo Adolfo Martín Small

    2011-01-01

    Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial. Palabras claves: Wegener, Granulomatosis, Vasculitis, Cáncer epidermoide.

  5. Wegener's Granulomatosis

    Science.gov (United States)

    ... to) Wegener’s, but no specific infection (bacterial, viral, fungal, or other) has been identified. How is Wegener’s ... ANCAs. Some patients with inflammatory bowel disease, rheumatoid arthritis, or SLE may have atypical P-ANCA test ...

  6. Association study with Wegener granulomatosis of the human phospholipase Cγ2 gene

    Directory of Open Access Journals (Sweden)

    Csernok Elena

    2005-02-01

    Full Text Available Abstract Background Wegener Granulomatosis (WG is a multifactorial disease of yet unknown aetiology characterized by granulomata of the respiratory tract and systemic necrotizing vasculitis. Analyses of candidate genes revealed several associations, e.g. with α(1-antitrypsin, proteinase 3 and with the HLA-DPB1 locus. A mutation in the abnormal limb mutant 5 (ALI5 mouse in the region coding for the hydrophobic ridge loop 3 (HRL3 of the phospholipaseCγ2 (PLCγ-2 gene, corresponding to human PLCγ-2 exon 27, leads to acute and chronic inflammation and granulomatosis. For that reason, we screened exons 11, 12 and 13 coding for the hydrophobic ridge loop 1 and 2 (HRL1 and 2, respectively and exon 27 of the PLCγ-2 protein by single strand conformation polymorphism (SSCP, sequencing and PCR/ restriction fragment length polymorphism (RFLP analyses. In addition, we screened indirectly for disease association via 4 microsatellites with pooled DNA in the PLCγ-2 gene. Results Although a few polymorphisms in these distinct exons were observed, significant differences in allele frequencies were not identified between WG patients and respective controls. In addition, the microsatellite analyses did not reveal a significant difference between our patient and control cohort. Conclusion This report does not reveal any hints for an involvement of the PLCγ-2 gene in the pathogenesis of WG in our case-control study.

  7. Estenosis subglótica y granulomatosis con poliangeítis (Wegener) en dos casos: Report of two cases

    OpenAIRE

    Massa,María; Nicholas C. Emery; Bosio, Martín; Finn,Bárbara C; Bruetman,Julio E; Young,Pablo

    2014-01-01

    Granulomatosis with polyangiitis (GPA) or Wegener's disease is characterized by a granulomatous vasculitis of the upper and lower airways and kidney. It involves the lower respiratory tract causing subglottic tracheal stenosis, which occurs in approximately 22% of patients. We report two females aged 40 and 52 years, admitted to the hospital with a subglottic tracheal stenosis. Their symptoms and management are reviewed. The frst patient responded to rituximab. The second patient required a t...

  8. Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome.

    Science.gov (United States)

    Andrassy, K; Erb, A; Koderisch, J; Waldherr, R; Ritz, E

    1991-04-01

    Patient survival and renal outcome were followed in 25 patients with biopsy confirmed Wegener's granulomatosis and renal involvement. Fourteen out of 25 patients required dialysis on admission, 11/25 patients did not. All patients were treated with a novel protocol comprising methylprednisolone and cyclophosphamide. The median follow-up observation was 36 months (12-113 months). With the exception of 1 patient (who died from causes not related to Wegener's granulomatosis) all patients are alive. Among the patients initially requiring dialysis (n = 14) 4 are in terminal renal failure after 0, 7, 21 and 38 months respectively. In the nondialysis group (n = 11) only 1 patient subsequently required chronic dialysis 30 months after clinical admission. Renal failure was due to non-compliance with immunosuppressive therapy in at least 2 patients. Percentage of obsolescent glomeruli and the degree of tubulointerstitial lesions, but not active glomerular lesions (crescents, necroses) predicted renal outcome. The major cause of renal functional impairment was relapse of Wegener's granulomatosis usually within 2 years after clinical remission. Therefore prolonged treatment with cyclophosphamide for at least 2 years after clinical remission is recommended. Two patients with initially negative immunohistology had a second renal biopsy which revealed de novo appearance of mesangial IgA deposits.

  9. Comprometimento da árvore respiratória na granulomatose de Wegener Laryngeal and tracheobronchial involvement in Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Ascedio Jose Rodrigues

    2012-04-01

    Full Text Available INTRODUÇÃO: A granulomatose de Wegener (GW é uma forma de vasculite sistêmica que envolve primariamente as vias aéreas superiores e inferiores e os rins. As manifestações mais frequentes nas vias aéreas são estenose subglótica e inflamações, estenoses da traqueia e dos brônquios. A visualização endoscópica das vias aéreas é a melhor ferramenta para avaliação, diagnóstico e manejo dessas alterações. OBJETIVOS: Descrever as alterações endoscópicas encontradas na mucosa das vias aéreas de um grupo de pacientes com GW submetido à broncoscopia no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP e relatar as intervenções terapêuticas broncoscópicas utilizadas em alguns casos. MÉTODOS: Foram estudados 15 pacientes com diagnóstico de GW provenientes do Ambulatório de Vasculites da Disciplina de Pneumologia do HC-FMUSP, encaminhados para a realização de broncoscopia no serviço de Endoscopia Respiratória do HC-FMUSP no período de 2003 a 2007. RESULTADOS: Dos 15 pacientes avaliados, 11 eram mulheres (73,33% com idade média de 34 ± 11,5 anos. Foram encontradas alterações das vias aéreas em 80% dos pacientes, e o achado endoscópico mais frequente foi estenose subglótica (n = 6. Realizou-se broncoscopia terapêutica em três pacientes com estenose subglótica e em outros três com estenose brônquica, todos apresentando bons resultados. CONCLUSÃO: A broncoscopia permite diagnóstico, acompanhamento e tratamento das lesões de vias aéreas na GW, constituindo-se um recurso terapêutico pouco invasivo em casos selecionados.INTRODUCTION: Wegener's granulomatosis (WG is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenoses. The endoscopic visualization of the airways is the best tool for assessing, diagnosing and

  10. A case of exorbitism in association with Wegener's granulomatosis with renal involvement.

    Science.gov (United States)

    Beji, S; Fatma, L Ben; Chebbi, A; Rais, L; Krid, M; Smaoui, W; Maiz, H Ben; Zouaghi, K; Moussa, F Ben

    2012-03-01

    Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

  11. [Subglottic stenosis in Wegener's granulomatosis--a diagnostic and therapeutic problem].

    Science.gov (United States)

    Wierzbicka, Małgorzata; Gaweqcki, Wojciech; Pastusiak, Tomasz; Szyfter, Witold

    2010-01-01

    Wegener's granulomatosis (WG) is a disease caused by necrotising vasculitis of small and middle blood vessels of upper and lower respiratory tract and kidneys of unknown etiology. ENT symptoms develop in more than 90% of patients and tracheobronchial involvement occurs in about 20% of patients, most often as a subglottic stenosis. Subglottic stenosis occurs usually as a late complication of disease, but sometimes it may be an early isolated symptom. It is usually irregular, no longer than 2-4 cm and affects mucosa and submucosa but sometimes also cartilage. The diagnosis is based on clinical symptoms, X-ray of the chest, urine analysis, c-ANCA detection and histological examination of the granulation from the stenosis. The main treatment of subglottic stenosis in WG is a causal immunosuppressive treatment, however an equally important is a preservation of respiratory tract passage, because a dominant symptom in this form of WG is problem with breathing or even dyspnoea. In this publication basing on literature review different methods of treatment of laryngotracheal stenosis and its importance in WG are described and discussed. In literature presently less invasive operations are recommended. The treatment of choice is endoscopic treatment with repeated dilatations and injections of steroid into the stenosis. In big stenosis in immunologically active disease patient sometimes requires tracheotomy and after remission of disease and maturation of the subglottic scar laryngotracheal resection can be considered.

  12. Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease.

    Science.gov (United States)

    Kalluri, R; Meyers, K; Mogyorosi, A; Madaio, M P; Neilson, E G

    1997-11-01

    A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the alpha 3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.

  13. Results of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    S. A. R. Nouraei

    2008-04-01

    Full Text Available Background: Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegener's Granulomatosis (WG. There is at present no consensus on the optimal management of this life-threatening condition. Objective: To assess the results of laryngo-tracheo-bronchoscopy, intralesional steroid therapy, laser surgery, and dilatation in managing obstructive tracheobronchial WG. Methods: Records of eighteen previously-untreated stridulous patients with obstructive tracheobronchial WG, treated between 2004 and 2006 were prospectively recorded on an airway database and retrospectively reviewed. Information about patient and lesion characteristics and treatment details were recorded. Treatment progress was illustrated using a timeline plot, and intervention-free intervals were calculated with actuarial analysis. Results: There were nine males and the average age at presentation was 40 (16 years [range 13–74]. There were thirteen patients with tracheal, and five patients with tracheal and bronchial lesions. The average tracheal lesion height was 8 (3 mm, located 23 (9 mm below the glottis. There were 1, 10 and 7 Myer-Cotton grade I, II and III lesions respectively. Mean intervention-free interval following minimally-invasive treatment was 26 (2.8 months. Following endobronchial therapy the median intervention-free interval was 22 months (p>0.8 vs. tracheal lesions. No patient required a tracheostomy or endoluminal stenting. Conclusions: Intralesional steroid therapy and conservative endoluminal surgery is an effective strategy for treating airway compromise due to active tracheal and bronchial WG, obviating the need for airway bypass or stenting. We recommend the combination of endotracheal dilatation, conservative laser surgery and steroid therapy as the standard of care for treating airway compromise due to obstructive tracheobronchial WG.

  14. Granulomatosis de Wegener Semejando Cáncer Epidermoide de Nasofaringe y Metástasis Pulmonares.

    Directory of Open Access Journals (Sweden)

    Gustavo Adolfo Martín Small

    2009-01-01

    Full Text Available La granulomatosis de Wegener (GW es una enfermedad idiopática, con posible componente autoinmune, que aparece generalmente en la quinta década de vida, caracterizándose por lesiones granulomatosas necrotizantes y vasculitis en vías aéreas y riñón. Paciente femenino de 54 años, quien desde Noviembre del 2008, presenta rinorrea, prurito y eritema en borde nasal inferior izquierdo, recibe antibióticos sin mejoría de los síntomas. En las radiografías torácicas, se observan dos radiopacidades redondeadas, de 4 cm de diámetro, sugestivas de lesiones tumorales en ambos campos pulmonares. La biopsia reporta cáncer epidermoide de alto grado, sospechándose primario en nasofaringe. Es remitida, por deterioro de condiciones, al Servicio de Neumonología del Hospital Universitario de Caracas el 18/03/2009, presentando disnea, tos productiva, placas purpúricas dolorosas en manos y pies, hipoacusia, hiperpigmentación del paladar duro, leucoplaquias y lesiones costrosas en lengua, insuficiencia renal (creatinina en 6,11 mg/dL y trombocitopenia. En TAC de tórax, se evidencian tumores mayores de 5 cm de diámetro, algunos con bordes bien definidos y otros mal delimitados con broncograma aéreo. Diagnostico definitivo de GW por serología. La GW con su afectación sistémica puede simular otras entidades como neoplasias de cabeza y cuello con metástasis pulmonares. Cuando las manifestaciones sistémicas son floridas debe sospecharse GW como diagnóstico diferencial.

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

    NARCIS (Netherlands)

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

    2004-01-01

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

  16. RELEVANCE OF CLASSIC ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODY (C-ANCA)-MEDIATED INHIBITION OF PROTEINASE 3-ALPHA-1-ANTITRYPSIN COMPLEXATION TO DISEASE-ACTIVITY IN WEGENER-GRANULOMATOSIS

    NARCIS (Netherlands)

    DOLMAN, KM; STEGEMAN, CA; VANDEWIEL, BA; HACK, CE; BORNE, AEGKV; KALLENBERG, CGM; GOLDSCHMEDING, R

    1993-01-01

    In the sera of patients with Wegener's granulomatosis (WG), C-ANCA can be detected that are directed against proteinase 3 (PR3). We have previously observed that C-ANCA interfere with PR3 proteolytic activity and with complexation of PR3 with its major physiologic inhibitor, alpha1-antitrypsin (alph

  17. The prevalence of the Staphylococcus aureus tst gene among community- and hospital-acquired strains and isolates from Wegener's Granulomatosis patients

    NARCIS (Netherlands)

    Deurenberg, Ruud H; Nieuwenhuis, Rutger F; Driessen, Christel; London, Nancy; Stassen, Frank R; van Tiel, Frank H; Stobberingh, Ellen E; Vink, Cornelis

    2005-01-01

    To allow rapid identification of toxic shock syndrome toxin-1 (TSST-1)-producing Staphylococcus aureus strains, a real-time PCR assay for the detection of the tst gene, which encodes TSST-1, was developed. The assay was applied to S. aureus isolates from patients with Wegener's Granulomatosis (WG),

  18. PREDOMINANCE OF IGG1 AND IGG4 SUBCLASSES OF ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES (ANCA) IN PATIENTS WITH WEGENERS GRANULOMATOSIS AND CLINICALLY RELATED DISORDERS

    NARCIS (Netherlands)

    BROUWER, E.; Tervaert, J.W.C.; HUITEMA, M.G.; VANDERGIESSEN, M.; Limburg, Piet; Kallenberg, Cees; Horst, G.

    1991-01-01

    In view of the supposed hypersensitivity, the elevated levels of IgE, and the occurrence of eosinophilia reported in Wegener's granulomatosis and related conditions, we studied the IgG subclass distribution of ANCA directed against a 29-kD serine protease and and myeloperoxidase (MPO) in 41 untreate

  19. [A case of rapidly progressive glomerulonephritis in the course of Wegener's granulomatosis].

    Science.gov (United States)

    Idasiak-Piechocka, I; Oko, A; Łochyńska, K; Woźniak, A; Czekalski, S

    2000-01-01

    Wegener's granulomatosis (WG) is characterized by granulomatous vasculitis of the respiratory tract and glomerulonephritis (GN). Prognosis of this disease is poor and about 20% of untreated patients die after one year from the onset. WG was recognized in 45-year-old patient on the basis of: 1) clinical symptoms (joint pain and swollen, purpura on the skin which appeared one week after respiratory tract infection, ulceration of the tonsils and lingula), 2) results of additional testing (X-chest-ray-infiltrates of both lungs), positive results of the cANCA (titre 1:640) and rapidly progressive renal failure [the increase of serum creatinine level (Pcr) from 123.7 to 707 mumol/l (1.4 to 8.0 mg/dl) during one week]. Renal biopsy revealed extracapillary GN (cellular crescents in 7 out of 8 glomeruli and scattered foci of fibrinoid necrosis of capillary walls in all). At the beginning of the treatment Pcr raised to 884 mumol/l (10 mg/dl) and the patient required hemodialysis. He was treated with methylprednisolone (M) at flash doses of 1000 mg/24 h by three days followed by 125 mg/24 h i.v.--because of peptic ulcer, with cyclophosphamide (C-150 mg/24 h p.p.), with trimetoprim/sulphametoxazole, with pentoxifylline and omeprazol. After six weeks of the treatment in the control kidney biopsy sclerotic changes in 10 out of 13 glomeruli and diffuse interstitial fibrosis were found. However, during the same time, we observed clinical remission of the disease and the decrease of Pcr to 176.8 mumol/l (2 mg/dl). The M dosis was reduced by 5 mg every weeks and the C dosis--to 50 mg (because of the increase of aminotransferase levels) After six months of the treatment Pcr was 132.6 mumol/l (1.5 mg/dl) and CANCA titer was 1:16. In this case of RPGN, despite off the progression of the morphological changes in the kidney, we obtained the clinical remission of the disease and significant decrease of Pcr level. These results suggest that aggressive treatment of WG is justified even in

  20. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

    DEFF Research Database (Denmark)

    Ozen, Seza; Pistorio, Angela; Iusan, Silvia M

    2010-01-01

    To validate the previously proposed classification criteria for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA)....

  1. High risk of pulmonary embolism and deep venous thrombosis but not of stroke in granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Obel, Niels; Baslund, Bo

    2014-01-01

    OBJECTIVE: To assess the incidence of stroke, pulmonary embolism (PE), and deep venous thrombosis (DVT) in granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: Patients diagnosed with GPA at a Danish tertiary care center during 1993-2011 were identified (n = 180). Each patient was matched...... with 19 population controls (n = 3,420). Information on hospitalizations for stroke, PE, and DVT was obtained from the Danish National Hospital Register. The occurrence of vascular events in the GPA cohort was compared with that in the control group by calculation of incidence rate ratios (IRRs). RESULTS......: The median duration of followup was 7.2 years (interquartile range 3.1-11.7 years) in the GPA cohort. Within the first 2 years following the diagnosis of vasculitis, the incidences of PE and DVT were substantially increased among the patients (IRR 25.7 [95% confidence interval (95% CI) 6.9-96] for PE and IRR...

  2. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Cabral, David A; Canter, Debra L; Muscal, Eyal

    2016-01-01

    OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency...... with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. RESULTS: In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time...... to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA...

  3. Tumour necrosis factor-alpha (TNF), lymphotoxin and TNF receptor levels in serum from patients with Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Jónasdóttir, O; Petersen, J; Bendtzen, K

    2001-01-01

    -R), and these receptors are often found in soluble forms (sTNF-R), which can modulate TNFalpha actions. To evaluate the clinical importance of the TNF family of cytokines, the serum levels of TNFalpha, TNFbeta, now termed lymphotoxin (LTalpha), and sTNF-R1 and sTNF-R2 were measured by ELISA in 8 patients with WG during......Wegener's granulomatosis (WG) is a systemic inflammatory disease with vasculitis as the key feature. Abnormal expression of tumour necrosis factor alpha (TNFalpha) is considered of prime pathogenic importance in several inflammatory diseases. The effects of TNFa are mediated by TNF receptors (TNF...... of the relative amounts of TNFalpha and sTNF-R indicated that TNFalpha was mostly bound to its soluble receptors. In WG, the serum levels of sTNF-R1 and sTNF-R2 were dramatically increased (p...

  4. On the Wegener granulomatosis associated region on chromosome 6p21.3

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    Csernok Elena

    2006-03-01

    Full Text Available Abstract Background Wegener granulomatosis (WG belongs to the heterogeneous group of systemic vasculitides. The multifactorial pathophysiology of WG is supposedly caused by yet unknown environmental influence(s on the basis of genetic predisposition. The presence of anti-neutrophil cytoplasmic antibodies (ANCA in the plasma of patients and genetic involvement of the human leukocyte antigen system reflect an autoimmune background of the disease. Strong associations were revealed with WG by markers located in the major histocompatibility complex class II (MHC II region in the vicinity of human leukocyte antigen (HLA-DPB1 and the retinoid X receptor B (RXRB loci. In order to define the involvement of the 6p21.3 region in WG in more detail this previous population-based association study was expanded here to the respective 3.6 megabase encompassing this region on chromosome 6. The RXRB gene was analysed as well as a splice-site variation of the butyrophilin-like (BTNL2 gene which is also located within the respective region. The latter polymorphism has been evaluated here as it appears as a HLA independent susceptibility factor in another granulomatous disorder, sarcoidosis. Methods 150–180 German WG patients and a corresponding cohort of healthy controls (n = 100–261 were used in a two-step study. A panel of 94 microsatellites was designed for the initial step using a DNA pooling approach. Markers with significantly differing allele frequencies between patient and control pools were individually genotyped. The RXRB gene was analysed for single strand conformation polymorphisms (SSCP and restriction fragment length polymorphisms (RFLP. The splice-site polymorphism in the BTNL2 gene was also investigated by RFLP analysis. Results A previously investigated microsatellite (#1.0.3.7, Santa Cruz genome browser (UCSC May 2004 Freeze localisation: chr6:31257596-34999883, which was used as a positive control, remained associated throughout the whole two

  5. Paquimeningite hipertrófica e parotidite: manifestações raras da granulomatose de Wegener Hypertrophic pachymeningitis and parotiditis: unusual ocurrences of the Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Ana Paula Monteiro Gomides

    2004-08-01

    Full Text Available A granulomatose de Wegener é uma vasculite sistêmica dos vasos de médio e pequeno calibre. Classicamente, há formação de granulomas com necrose no trato respiratório e glomerulonefrite necrosante. Embora seu acometimento mais comum envolva o trato respiratório superior, pulmões e rins, uma vasta gama de manifestações em vários órgãos e tecidos é descrita. Relatamos o caso de um paciente que, paralelamente às manifestações típicas da doença, desenvolveu algumas alterações raras como a parotidite e a paquimeningite hipertrófica. Diante de quadros graves, atípicos e/ou refratários ao tratamento convencional, torna-se necessário o aprofundamento no estudo e uso de novas armas terapêuticas.The wegener's granulomatosis is a systemic angiitis of small and medium caliber vessels. Normally, it is characterized by the formation of granulomas with necrosis in the respiratory system and necrosing glomerulonephritis. even though it is more common in the upper respiratory system, in the lung and in the kidney, a great range of manifestation in other different organs and tissues can be described. we report the case of a patient who, in parallel with the typical symptoms of this disease, developed some rare alteration such as the parotiditis and the hypertrophic pachymeningitis. in face of serious situations, severe disease, atypical or non responsive to conventional treatments, it is necessary a more detailed study of the case and the use of new therapeutic drugs.

  6. Progressive renal disease despite immunosuppressive therapy in a patient with Wegener s granulomatosis.

    NARCIS (Netherlands)

    Klein, I.; Vervoort, G.; Steenbergen, E.; Wetzels, J.

    2008-01-01

    We present a patient with Morbus Wegener and crescentic glomerulonephritis. Treatment with cyclophosphamide and prednisolone resulted in the disappearance of signs and symptoms of systemic inflammation. However, renal function deteriorated. Renal biopsy showed evidence of continuing capillary necros

  7. Perda auditiva neurossensorial em pacientes com granulomatose de Wegener: relato de três casos e revisão de literatura Neurosensory hearing loss in patients with Wegener's granulomatosis: a report of three cases and literature review

    Directory of Open Access Journals (Sweden)

    Ana Paula Monteiro Gomides

    2006-06-01

    Full Text Available A granulomatose de Wegener é uma vasculite sistêmica que acomete vasos de pequeno e médio calibres. As manifestações clássicas ocorrem no trato respiratório superior, inferior e rins, mas outros órgãos podem ser envolvidos. O sistema auditivo pode ser freqüentemente acometido nas suas várias porções (orelha externa, média e/ou interna com manifestações diversas. A perda auditiva neurossensorial pode ser conseqüência de alterações na orelha interna e deve ser precocemente reconhecida. O tratamento rápido e efetivo pode evitar danos irreversíveis. Descrevemos três casos de granulomatose de Wegener e hipoacusia sensorial, com respostas diferentes ao tratamento.Wegener's granulomatosis is a primary systemic vasculitis that affects small and medium-sized vessels. The classical manifestations occur in the upper and lower respiratory tract and in the kidneys, but other organs may be involved. The auditory system can be frequently affect in its various portions (outer, middle and/or inner ear, showing different manifestations. Neurosensory hearing loss might be a consequence of changes in the inner ear and should be recognized early. Rapid and effective treatment may prevent irreversible damage. We report herein three cases of Wegener's granulomatosis and sensory hypoacusis, which responded differently to treatment.

  8. Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001

    OpenAIRE

    Cisternas M,Marcela; Soto S,Lilian; Jacobelli G,Sergio; Marinovic M,María Angélica; Vargas B,Alex; Sobarzo V,Elizabeth; Saavedra M,Jorge; Chauan I,Karina; Meléndez T,Gabriela; Foster B,Carolina; Pacheco R,Daniel; Wainstein G,Eduardo

    2005-01-01

    Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 5...

  9. Epidemiology and etiology of wegener granulomatosis, microscopic polyangiitis, churg-strauss syndrome and goodpasture syndrome: vasculitides with frequent lung involvement.

    Science.gov (United States)

    Gibelin, Aude; Maldini, Carla; Mahr, Alfred

    2011-06-01

    This review focuses on the epidemiological characteristics and etiologies of four primary systemic vasculitides with frequent lung involvement, namely Wegener granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS), and Goodpasture syndrome (GPS). Elucidation of the mechanisms underlying these vasculitides with frequent lung involvement is complicated by their rarity, which hampers the undertaking of large-scale studies; difficulties in classification; and their multifaceted clinical presentations, which infer the existence of several etiologic pathways. Notwithstanding, epidemiological research showed some evidence for international, interethnic, and temporal variations of the frequencies of these four vasculitides; led to the identification of several genetic and environmental risk factors; and provided insight on the extent to which genes and environment might contribute to their development. Available data support the concept that WG, MPA, CSS, and GPS have unique and shared risk determinants. Although the precise causes of these vasculitides are not yet fully understood and the development of prevention strategies is out of our reach at present, current knowledge enables the formulation of etiologic hypotheses to provide caregivers and their patients with valuable information on the nature of these rare entities. © Thieme Medical Publishers.

  10. Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy

    DEFF Research Database (Denmark)

    Hruskova, Zdenka; Stel, Vianda S; Jayne, David;

    2015-01-01

    for microscopic polyangiitis (prevailing in Southern Europe). Kidney transplantation was performed in 558 (22.2%) patients with vasculitis. The 10-year probability for survival on RRT after day 91 was 32.5% (95% CI, 29.9%-35.1%) in patients with vasculitis. Survival on RRT after day 91 did not differ between AAV...... manifestations, treatment, and relapses. CONCLUSIONS: Geographical differences in the incidence of RRT for kidney failure due to granulomatosis with polyangiitis and microscopic polyangiitis copied their distribution in the general population. Overall survival on RRT after day 91 for patients with AAV...

  11. Mastoidite e paralisia facial como manifestações iniciais de Granulomatose de Wegener Mastoiditis and facial paralysis as initial manifestations of Wegener's Granulomatosis

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    André Souza de Albuquerque Maranhão

    2012-04-01

    Full Text Available A Granulomatose de Wegener (GW é caracterizada por granulomas necrotizantes e vasculite. Sem tratamento a doença tem prognóstico pobre com índice de mortalidade de 90% em 2 anos. Diversos autores citam as manifestações otológicas no curso da GW, entretanto não é especificado em que momento da doença elas apareceram, isto é, se como manifestação inicial ou subsequente a outros achados. Objetivo: Descrever três casos confirmados de GW que apresentaram inicialmente mastoidite e evoluíram com paralisia facial periférica (PFP. Material e Método: Estudo de série de casos. Pacientes diagnosticados com GW que apresentaram inicialmente manifestações otológicas são descritos. Resultados: Os três casos descritos abriram o quadro com otalgia, otorreia e hipoacusia unilateral, associada a paralisia facial periférica ipsilateral. Tiveram resposta inadequada aos tratamentos instituídos o que motivou uma investigação de outras doenças associadas. Nessas circunstâncias, detectaram-se títulos positivos de ANCA-C em todos pacientes, confirmando-se o diagnóstico de GW, após período variável de investigação. Institui-se o tratamento para GW observando-se melhora do quadro clínico, regressão da PFP e melhora parcial dos limiares auditivos. Conclusão: Complicações de otites médias agudas (mastoidite e PFP refratárias as terapêuticas habituais impõem a investigação de doenças associadas e a GW deverá ser pesquisada para que se possa fazer o diagnóstico o mais precocemente possível, alterando desta forma o prognóstico destes pacientes.Wegener's Granulomatosis (WG is characterized by necrotizing granulomas and vasculitis. If left untreated, the prognosis is poor - a 90% mortality rate within 2 years. Several authors have described the otologic manifestations of WG; these authors, however, have not mentioned the stage of the disease in which these findings present - whether as initial manifestations or subsequent to

  12. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

    DEFF Research Database (Denmark)

    Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela;

    2010-01-01

    To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria....

  13. Vasculitis de Wegener: diferentes presentaciones pulmonares en el diagnóstico inicial y durante la evolución de la enfermedad Wegener granulomatosis: different pulmonary abnormalities at initial diagnostic and during the course of disease

    Directory of Open Access Journals (Sweden)

    Marta Hernandorena González

    2009-09-01

    Full Text Available La enfermedad de Wegener es una vasculitis granulomatosa necrotizante, con afectación característica del tracto respiratorio superior, pulmones y riñones. El 90% de los pacientes tienen afectación pulmonar y los hallazgos radiográficos más frecuentes son nódulos o masas bilaterales múltiples que pueden mostrar cavitación. También se puede presentar como consolidación lobar o segmentaria o como hemorragia pulmonar difusa. El reconocimiento precoz de las alteraciones específicas es crítico para realizar un diagnóstico apropiado y para reducir la morbi-mortalidad. Con el objetivo de lograr una mejor caracterización de las distintas manifestaciones torácicas de esta entidad presentamos cuatro casos que muestran sus distintas manifestaciones radiológicas.Wegener granulomatosis is a necrotizing granulomatous vasculitis that affects the upper respiratory tract, lungs, and kidneys. At least 90% of patients have pulmonary involvement, and the typical radiographic findings are bilateral multiple nodules or masslike lesions, some of which show cavitation. Other frequent findings are consolidations or diffuse pulmonary hemorrhage. Early recognition of specific abnormalities is critical for appropriate intervention and reduced morbidity. To better characterize the diversity of thoracic manifestations in this disease we present four cases to show different radiologic findings in this population.

  14. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Cryopyrin-Associated Autoinflammatory Syndrome (CAPS) (Juvenile) Dermatomyositis (Juvenile) Familial Mediterranean Fever (Juvenile) Fibromyalgia Giant Cell Arteritis Glucocorticoid-induced Osteoperosis ...

  15. Granulomatosis with Polyangiitis (Wegener's)

    Science.gov (United States)

    ... Pharyngitis, Adenitis Syndrome (Juvenile) Polymyalgia Rheumatica Psoriatic Arthritis Raynaud's Phenomenon Reactive Arthritis Rheumatoid Arthritis Scleroderma Sjogren's Syndrome Spinal Stenosis Spondyloarthritis Systemic Lupus Erythematosus (Juvenile) Takayasu's ...

  16. Estenose subglótica como manifestação clínica da granulomatose de Wegener em adolescentes: relato de caso e revisão de literatura Subglottic stenosis as a clinical manifestation of Wegener's granulomatosis in adolescents: report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Márcia C. Machado

    2003-10-01

    Full Text Available A Granulomatose de Wegener é uma patologia caracterizada por vasculite sistêmica e inflamação granulomatosa necrotizante que compromete o trato respiratório alto, pulmões e rins, cujas manifestações iniciais, na maioria das vezes, ocorre em maiores de 20 anos de idade. As queixas clínicas otorrinolaringológicas estão, com freqüência, presentes nas fases iniciais da doença, sendo rinite, sinusopatia de repetição e epistaxe as mais comuns. A estenose subglógica parece estar correlacionada com a Granulomatose de Wegener em adolescentes. Relatamos o caso de uma paciente portadora de Granulomatose de Wegener cujo início dos sintomas foi na infância, tendo evoluído com estenose laríngea durante o curso da doença.Wegener's Granulomatosis is a well-characterized systemic vasculitis and necrotising granulomatous inflammation of the upper, lower respiratory tracts and the kidneys. The initial manifestations of the disease usually occur in patients over than twenty years old. The otolaryngologic symptoms like rhinorrhea, recurrent sinusitis and epistaxis are commonly present in early course of the disease. It seems that subglottic stenosis is correlated to Wegener's granumatosis in adolescents. We describe a case of a patient that developed subglottic stenosis associated with Wegener's granulomatosis in childhood during the course of the disease.

  17. Clinical analysis of 10 children with Wegener granulomatosis%儿童韦格纳肉芽肿10例临床分析

    Institute of Scientific and Technical Information of China (English)

    袁林; 陈植; 张桂菊; 沈颖

    2009-01-01

    Objective To investigate the characteristics of Wegener granulomatosis in children and to improve its recognition. Methods Ten pediatric patients with Wegener granulomatosis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment, etc. Results All children had clinical evidence of upper respiratory tract and lungs" involvement; kidney lesion occurred in 6 cases(6/10); joints, skin, eyes and nervous system were also involved with different degree, cANCA (PR3) showed positive in 8 cases; Pathological examination (1 kidney sample, 2 nasal mucosa samples, 2 skin samples) showed granulomatous vasculitis and crescents were found in renal biopsy; 7 cases were treated with corticosteroid plus cyclophosphamide, 1 case was treated with corticosteroid plus methotrexate, and improvement were seen in all of them. Conclusions Wegener granulomatosis in children have diverse clinical manifestations and clinical diagnosis is difficult. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Treatment with corticosteroid and immunosuppressive can achieve good outcome.%目的 分析儿童韦格纳肉芽肿的特点,提高对该病的认识.方法 对10例韦格纳肉芽肿患儿的临床表现、辅助检查、病理结果、治疗等资料进行总结分析.结果 10例患儿中上呼吸道、肺脏受累10例,肾脏受累6例,关节、皮肤、眼及神经系统等也有不同程度受累.实验室检查胞浆型抗中性粒细胞胞浆抗体(cANCA)阳性8例;5例行病理检查(肾脏1例,鼻黏膜2例,皮肤2例),均表现血管炎和(或)肉芽肿性改变,肾活检有新月体形成.7例患儿给予糖皮质激素(甲基泼尼松龙冲击)联合环磷酰胺治疗,1例进行糖皮质激素联合氨甲蝶呤治疗,患儿临床表现均有不同程度的改善.结论 儿童韦格纳肉芽肿临床表现多样,误诊率高;主要累及呼吸道及肾脏,ANCA检查有特

  18. Clinical pathological of Wegener's granulomatosis in middle-aged and elderly patients%中老年人Wegener's肉芽肿病的临床病理观察

    Institute of Scientific and Technical Information of China (English)

    方芳; 乔旭柏; 蒲纯; 庞建欣; 刘东戈

    2013-01-01

    目的 探讨Wegener's肉芽肿病(WG)的临床病理学特点,提高对本病的认识.方法 回顾性分析1999年2月至2012年7月我院诊治的21例WG患者的临床、病理资料,其中男11例,女10例;年龄45~76岁,平均58.1岁.34个(包括2例尸检)不同部位的标本常规取材、制片,并进行苏木精-伊红(HE)、组织化学染色,13例肾穿标本全部行免疫荧光染色和电镜检查,观察病理形态特征. 结果 从初发到确诊的时间为24 d至11.0个月,平均为5.3个月.眼、鼻和涎腺是本病病初最容易受累的部位,共11例(52.4%);皮肤受累3例(14.3%),17例(81.0%)累及肺脏,15例(71.4%)累及肾脏.18例检查抗中性粒胞浆抗体(ANCA),13例(72.2%)胸型ANCA (cANCA)阳性;3例(16.7%)核周型ANCA(p-ANCA)阳性;2例(11.1%)ANCA阴性.病理学主要表现:(1)7种血管炎,依次为毛细血管炎、急性血管炎、慢性血管炎、坏死性纤维素性血管炎、坏死性肉芽肿性血管炎、非坏死性肉芽肿性血管炎、瘢痕性血管炎;(2)4种肉芽肿性炎,依次为散在分布的巨细胞、栅栏状排列的组织细胞、松散的小肉芽肿及微脓肿周围车轮状排列的组织细胞;(3)2种实质的变性坏死,地图状坏死、中性粒细胞微脓肿.2例尸检3类13种形态的表现均可观察到,活检小标本可见不同种类的形态变化和弥漫性肺出血等次要表现. 结论 Wegener's肉芽肿病有复杂的病理形态学的变化谱系,主要表现为血管炎、肉芽肿性炎和实质的坏死.%Objective To study the clinical pathological features of Wegener's granulomatosis (WG) in middle-aged and elderly patients,and enhance understanding of this disease.Methods Totally 21 patients with WG (11 males,10 females,aged 45 to 76 years,mean age 58.1 years) in our hospial from February 1999 to July 2012 were selected.The clinical and pathological data of WG patients were retrospectively analyzed.34 biopsies including 2 autopsies

  19. Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

    Directory of Open Access Journals (Sweden)

    Letícia Stahelin

    2012-06-01

    Full Text Available O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC, que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL, which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG. We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been

  20. Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study.

    Science.gov (United States)

    Cabral, David A; Canter, Debra L; Muscal, Eyal; Nanda, Kabita; Wahezi, Dawn M; Spalding, Steven J; Twilt, Marinka; Benseler, Susanne M; Campillo, Sarah; Charuvanij, Sirirat; Dancey, Paul; Eberhard, Barbara A; Elder, Melissa E; Hersh, Aimee; Higgins, Gloria C; Huber, Adam M; Khubchandani, Raju; Kim, Susan; Klein-Gitelman, Marisa; Kostik, Mikhail M; Lawson, Erica F; Lee, Tzielan; Lubieniecka, Joanna M; McCurdy, Deborah; Moorthy, Lakshmi N; Morishita, Kimberly A; Nielsen, Susan M; O'Neil, Kathleen M; Reiff, Andreas; Ristic, Goran; Robinson, Angela B; Sarmiento, Angelyne; Shenoi, Susan; Toth, Mary B; Van Mater, Heather A; Wagner-Weiner, Linda; Weiss, Jennifer E; White, Andrew J; Yeung, Rae S M

    2016-10-01

    To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with

  1. Clinical analysis of patients with Wegener's granulomatosis complicated with pulmonary infection%韦格纳肉芽肿病合并肺部感染的临床分析

    Institute of Scientific and Technical Information of China (English)

    曾平; 张国华; 苏金梅; 张文; 曾小峰

    2011-01-01

    目的 分析韦格纳肉芽肿病合并肺部感染的临床特点和危险因素.方法 回顾性分析北京协和医院1998-2009年收治的韦格纳肉芽肿病合并肺部感染患者的临床资料.采用t检验和Fisher检验进行统计分析.结果 92例韦格纳肉芽肿病患者中合并肺部感染27例(29%).其中首次就诊即合并肺部感染7例占26%,确诊韦格纳肉芽肿病0~6个月内发生肺部感染12例(44%),确诊韦格纳肉芽肿病6个月以上发生肺部感染8例(30%).肺部感染表现为咳嗽、咯痰(89%),咯血(63%),发热、乏力(56%),胸闷、胸痛(33%).主要为细菌感染(59%),真菌感染(37%),结核分枝杆菌感染(37%).WG合并肺部感染组有鼻窦感染( P=0.01)、低蛋白血症(P=0.03)、低免疫球蛋白血症(P=0.007)、接受激素冲击治疗(P=0.002)的比例高于无肺部感染组.结论 韦格纳肉芽肿病合并肺部感染0~6个月内发生率高,临床上表现为咳嗽、咯痰,病原学检查细菌、真菌、结核感染多见,与鼻窦感染、低蛋白血症、低免疫球蛋白血症、接受激素冲击治疗有关.%Objective To investigate the clinical features and risk factors of patient with Wegener's granulomatosis complicated with pulmonary infection.Methods Patients with Wegener's granulomatosis admitted to our hospital in the past 11 years were retrospectively analyzed.Comparisons between groups were performed by t tests or Fisher test.Results Pulmonary infection occurred in 27 cases with an incidence rate of 29%.Twenty-six percent of pulmonary infections occurred at the initial diagnosis,and 44% occurred within 6 months,while 30% occurred later than 6 months.The clinical manifestations of pulmonary infection were productive cough (89%),hemoptysis (63%),fever and fatigue (56%),chest pain and pactoralgia (33%).The most common causative pathogen were bacteria(59% ),fungi(37% ),and tubercle bacillus(37% ).Sinus infection (P=0

  2. IL-17R expression on immune cells in peripheral blood of Wegener's granulomatosis patients%IL-17R在韦格纳肉芽肿患者外周血免疫细胞的表达

    Institute of Scientific and Technical Information of China (English)

    姜波; 王红; 刘布骏

    2011-01-01

    Objective To assess whether the receptor of IL- 17 (IL- 17R) expression on peripheral blood immune cells including B lymphocytes and plasma cells of Wegener's granulomatosis (WG) patients is abnormal. Methods Flow cytometry was used to analyze the expression of IL - 17R protein on peripheral blood immune cells of health control (HC) and WG patients. Results There was no difference of IL- 17R expression on PMN, monocytes, total lymphocytes between WG patients and HC. However, IL - 17R expression on B lymphocytes (P - 0.039) and plasma cells (P = 0.043) were much higher in WG patients compared with HC. There was no difference of IL- 17R expression on these cells between active and inactive WG, cyclophosphamide (Cyc) and methotrexate (MTX) treated WG. Conclusion IL~ 17R expression on B lymphocytes and plasma cells are significantly increased in WG patients, but normal on PMN and monocytes.%目的 检测韦格纳肉芽肿(WG)患者外周血免疫细胞特别是B淋巴细胞和浆细胞上IL-17受体(IL-17R)的表达有无异常.方法 流式细胞仪检测正常人(HC)及WG患者外周血免疫细胞表面IL-17R蛋白的表达.结果 IL-17R在WG患者外周血中性粒细胞(PMN)、单核细胞(Mono)、总淋巴细胞(Lym)上的表达与正常人无显著差异,但在B淋巴细胞(CD19+ CD20+ )(P<0.05)和浆细胞(CD19+CD38+)(P<0.05)上的表达显著高于正常人;活动期与非活动期患者、环磷酰胺(Cyc)和甲氨蝶呤(MTX)治疗组患者1L-17R的表达无显著差异.结论 IL-17R在WG患者外周血B淋巴细胞和浆细胞上的表达显著升高,但在中性粒细胞和单核细胞上的表达无明显异常.

  3. Prevalência das manifestações clínicas iniciais da granulomatose de Wegener no Brasil: relato de seis casos e revisão da literatura Wegener's granulomatosis: prevalence of the initial clinical manifestations - report of six cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Carlos Ewerton Maia Rodrigues

    2010-04-01

    Full Text Available OBJETIVOS: Descrever as manifestações clínicas iniciais da Granulomatose de Wegener (GW diagnosticada no Brasil. PACIENTES E MÉTODOS: Análise retrospectiva de seis prontuários do Serviço de Reumatologia do Hospital Geral de Fortaleza (HGF, assim como a realização de um levantamento bibliográfico dos casos de GW descritos no Brasil obtidos dos bancos de dados LILACS, SciELO e MEDLINE. RESULTADOS: O estudo identificou 49 pacientes; 15 (31% do sexo masculino e 34 (69% do sexo feminino. A forma sistêmica ocorreu em 35 pacientes (73%: 28 adultos, cinco crianças e dois adolescentes. A doença limitada ocorreu em 13 adultos e uma criança. A média da idade adulta no início da doença foi de 42,2 anos (18 a 65 anos. O quadro clínico agudo, com sintomas há menos de três meses do diagnóstico, ocorreu em 41% (20/49 da casuística e a forma insidiosa, em 59% (29/49 dos pacientes. A prevalência das manifestações clínicas iniciais nos adultos com doença sistêmica (n = 28 foi 64% (18/28 das vias aéreas superiores (VAS, 36% (10/28 pulmonares, 18% (5/28 renais, 25% (7/28 oculares, 11% (3/28 cutâneas, 25% (7/28 musculoesqueléticas e 7% (2/28 neurológicas. Na forma limitada do adulto (n = 13, os sintomas prevalentes foram 84% (11/13 VAS, 23% (3/13 oculares e 15% (2/13 pulmonares. CONCLUSÃO: No Brasil, a prevalência das manifestações clínicas iniciais da GW foi semelhante aos resultados da literatura. A falta de especificidade dos sintomas pode retardar o diagnóstico na forma insidiosa da doença e aumentar a morbimortalidade das formas agudas.OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF, as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study

  4. Trombose em artérias pulmonares pequenas e médias em granulomatose de Wegener: um estudo com microscopia confocal por varredura a laser Thrombosis in small and medium-sized pulmonary arteries in Wegener's granulomatosis: a confocal laser scanning microscopy study

    Directory of Open Access Journals (Sweden)

    Alfredo Nicodemos Cruz Santana

    2010-12-01

    Full Text Available OBJETIVO: A granulomatose de Wegener (GW pode causar dano nas células endoteliais e fenômenos tromboembólicos. Entretanto, poucos estudos analisaram a microcirculação pulmonar - artérias pulmonares de pequeno/médio calibre (APPMC - em pacientes com GW. O objetivo deste estudo foi quantificar trombos de fibrina em amostras de APPMC de pacientes com GW. MÉTODOS: Analisamos 24 APPMC de seis pacientes com GW e 16 APPMC de quatro pacientes controles sem WG. Utilizamos CD34 para a marcação do endotélio em todas as amostras e microscopia confocal a laser para detectar trombos de fibrina intravasculares. Calculamos a área total do vaso, a área livre do lúmen e a área trombótica. RESULTADOS: A média da área total do vaso foi similar no grupo GW e no grupo controle (32.604 µm² vs. 32.970 µm², p = 0,8793. Trombos foram identificados em 22 das 24 APPMC (91,67% no grupo GW, e em nenhuma do grupo controle (p OBJECTIVE: Wegener's granulomatosis (WG can cause endothelial cell damage and thromboembolic events. Nevertheless, there have been few studies on the pulmonary microcirculation-small and medium-sized pulmonary arteries (SMSPA-in patients with WG. The objective of this study was to quantify fibrin thrombi in the SMSPA of patients with WG. METHODS: We analyzed 24 SMSPA samples collected from six patients with WG and 16 SMSPA samples collected from four patients without WG. In all samples, we used the endothelial cell marker CD34 and confocal laser scanning microscopy in order to detect intravascular fibrin thrombi. We calculated the total vessel area, the free lumen area, and the thrombotic area. RESULTS: The mean total vessel area was similar in the WG and control groups (32,604 µm² vs. 32,970 µm², p = 0.8793. Thrombi were present in 22 (91.67% of the 24 WG group samples and in none of the control group samples (p < 0.0001; OR = 297; 95% CI: 13.34-6,612. The mean thrombotic area was greater in the WG group samples than in the

  5. Adolescente femenino con granulomatosis de Wegener fulminante

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    S C Scheffler-Mendoza

    2017-01-01

    Full Text Available Adolescente femenino de 13 años de edad, originaria de Guerrero, grupo étnico mixteco, padres analfabetos y con poco entendimiento del español. Sin otros antecedentes de importancia para el padecimiento. Inició un mes previo con la aparición de una pápula hipercrómica en párpado superior derecho que en los 10 días previos al ingreso aumentó de volumen generando proptosis. Al interrogatorio se negó dolor, lagrimeo, fiebre, o pérdida de peso. Ingresó con fiebre (39.0°C, palidez generalizada, proptosis derecha con exposición de córnea y conjuntiva, eritema periorbitario, movimientos oculares y agudeza visual disminuidos (i.e. contaba dedos a 1 m, pupila con tendencia a midriasis, hiperreactividad a la luz, y fondo de ojo con papila hiperémica edematosa y tortuosidad peripapilar. El resto de exploración no mostró datos relevantes. Se hospitalizó para iniciar tratamiento antimicrobiano parenteral de amplio espectro (i.e. ceftriaxona y vancomicina y abordar proptosis unilateral.

  6. Sclerosing Wegener's granulomatosis in the orbit

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Heegaard, Steffen; Prause, Jan Ulrik

    2008-01-01

    and from two enucleated eyes. RESULTS: All three patients had longstanding inflammation in the orbit and involvement of the paranasal sinuses. Proptosis, impaired ocular motility and reduced visual acuity dominated the clinical picture. All histopathological specimens featured granulomatous inflammation...

  7. Orofacial Granulomatosis.

    Science.gov (United States)

    Al-Hamad, Arwa; Porter, Stephen; Fedele, Stefano

    2015-07-01

    Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder of the orofacial region. It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. Etiopathogenesis is unknown. A range of systemic granulomatous disorders, including Crohn disease and sarcoidosis, may cause orofacial manifestations that cannot be distinguished from those of OFG. Treatment of OFG has proven difficult and unsatisfactory, with no single therapeutic model showing consistent efficacy in reducing orofacial swelling and mucosal inflammation.

  8. Orofacial granulomatosis

    Directory of Open Access Journals (Sweden)

    Srinivas Kandula

    2016-01-01

    Full Text Available Orofacial granulomatosis (OFG is a rare disease, usually presenting as a persistent swelling of the soft tissues in the orofacial region, predominantly lips (cheilitis granulomatosa. The treatment of OFG can be challenging as frequent recurrences are very common. Here, we present a case of an Asian female patient with persistent swelling of the upper lip from the past 6 months. After establishing the diagnosis, intralesional triamcinolone injection (10 mg on a weekly schedule was planned for a period of 6 weeks. Complete resolution of the lesion was observed. This article presents a unique case of cheilitis granulomatosa (CG with a brief highlight on the various treatment modalities and a structured algorithm discussing the various differential diagnoses, aiding in formulating an accurate diagnosis and effective treatment.

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

    Directory of Open Access Journals (Sweden)

    Cristina La Rosa

    2013-01-01

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

  10. [Pulmonar pseudotumor in granulomatosis with polyangiitis (GPA). Pulmonary cancer and/or GPA? Diagnostic implications of pulmonary nodules].

    Science.gov (United States)

    Horta-Baas, Gabriel; Meza-Zempoaltecatl, Esteban; Pérez-Cristóbal, Mario; Barile-Fabris, Leonor Adriana

    2016-01-01

    Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a systemic necrotizing vasculitis, which affects small and medium sized blood vessels and is often associated with cytoplasmic anti-neutrophil cytoplasmic antibodies (ANCA). Inflammatory pseudotumor is a rare condition characterized by the appearance of a mass lesion that mimics a malignant tumor both clinically and on imaging studies, but that is thought to have an inflammatory/reactive pathogenesis. We report a patient with a GPA which was originally diagnosed as malignancy.

  11. Alfred Wegener - en ilisimatoorsuaq

    DEFF Research Database (Denmark)

    Andersen, Jan

    2007-01-01

    Den naturvidenskabelige grønlandsforskning i begyndelsen af 1900-tallet skulle ikke involvere sig med Grønland. Dens projekt handlede om naturen - ikke kulturen. Wegener er kendt for sin kontinentaldriftsteori, der ofte fremstilles som et resultat af Danmark-ekspeditionens videnskabelige arbejde....... Da Wegener som medlem af ekspeditionen opholdt sig i Nordøstgrønland var tanken om kontinentaldrift imidlertid endnu ikke faldet ham ind. Vi har hans egne ord for, at ideen først opstod i 1910....

  12. Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Baslund, Bo; Wiencke, Anne Katrine; Rasmussen, Niels

    2012-01-01

    by CT-scan before treatment (3 had bilateral and 7 unilateral orbital involvement). Orbital symptoms at study baseline included pain, pressure sensation behind the eyes, epiphora, diplopia, and affection of the visual acuity. Nine out of ten patients experienced subjective improvement. Four patients...

  13. Treatment of orbital inflammation with rituximab in Wegener's granulomatosis

    DEFF Research Database (Denmark)

    Baslund, Bo; Wiencke, Anne Katrine; Rasmussen, Niels

    2012-01-01

    inflammation. All patients were treated with 1000 mg of rituximab administered twice with an interval of 14 days between the infusions. Six months after therapy, a physical examination and a control computerised tomography (CT) scan was performed. RESULTS: All patients had orbital inflammation demonstrated...... by CT-scan before treatment (3 had bilateral and 7 unilateral orbital involvement). Orbital symptoms at study baseline included pain, pressure sensation behind the eyes, epiphora, diplopia, and affection of the visual acuity. Nine out of ten patients experienced subjective improvement. Four patients...... (seven eyes) with visual impairment responded to therapy, and the improvement in visual acuity was sustained throughout follow-up (median duration of follow-up: 17 months; range: 6-18 months). At the time of the control CT-scan, size-reduction of the orbital mass was observed in two patients, while...

  14. Cancer preceding Wegener's granulomatosis: a case-control study

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Sørensen, Inge Juul

    2009-01-01

    .4; 95% CI 1.1, 38) based on two patients, who developed testis cancer >10 years before WG. The overall prevalence of malignancies diagnosed skin cancer occurred with an increased prevalence within this time...... interval (OR 4.0; 95% CI 1.4, 12). CONCLUSIONS: We did not find clear evidence of an increased prevalence of preceding cancer in our WG cohort, indicating that shared risk factors are of minor importance for the excess of malignancies that occur in WG patients after the vasculitis diagnosis. Furthermore...... randomly for each patient from the Danish Central Population Register. Information on malignancies was obtained through the Danish Cancer Registry. Occurrence of malignancies before WG diagnosis among patients and before WG diagnosis of their matched case among controls (reference date) was compared...

  15. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness.

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il; Yeo, Seung Geun

    2016-04-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

  16. Coexistence of granulomatosis with polyangiitis (GPA) and Crohn's disease or multiorgan manifestation of the same disease?

    Science.gov (United States)

    Jóźwiak, Lucyna; Ławnicka, Izabela; Książek, Andrzej

    2016-01-01

    Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis of unknown aetiology, often related to the antineutrophil cytoplasmic antibody (ANCA). GPA was previously named Wegener's granulomatosis (WG). The disease frequently has multisystemic presentation, targeting mainly the respiratory tract and kidneys, but gastrointestinal involvement is uncommon. Crohn's disease (CD) is an inflammatory bowel disease (IBD) with many extraintestinal manifestations. Clinically, symptoms of WG and CD can mimic each other. In this paper a case of GPA manifested initially by severe multiorgan damage including colitis, regarded to be coexistent CD, is presented. The case illustrates the difficulties in establishing the diagnosis when symptoms of the diseases mimic each other.

  17. Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

    Science.gov (United States)

    Kim, Sang Hoon; Jung, A Ra; Kim, Su Il

    2016-01-01

    Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab. PMID:27144236

  18. GRANULOMATOSIS WITH POLYANGIITIS, WHICH IS PATHOGENETICALLY ASSOCIATED WITH ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES: CLINICAL FEATURES

    Directory of Open Access Journals (Sweden)

    Tatiana Valentinovna Beketova

    2012-01-01

    Full Text Available The author studied in detail the specific features of the clinical manifestations reflecting a granulomatous inflammatory response and necrotizing polyangiitis, by using her experience in following up 70 patients with granulomatosis and polyangiitis (Wegener's, and analyzed their early symptoms, premorbid background, and possible predictors. Granulomatous inflammation and its related clinical manifestations (primarily orbital pseudotumor and subfold granuloma of the larynx was demonstrated to tend over time to occupy a dominant place in the clinical picture of the disease while the proportion of the symptoms apparently related to necrotizing vasculitis was generally decreased. Interpretation of mechanisms for the development of granulomatosis with polyangiitis (Wegener's may be of value for the further elaboration of an optimal treatment strategy.

  19. Hemorrhagic acneiform lesions in a teenager as the initial presentation of granulomatosis with polyangiitis.

    Science.gov (United States)

    Dosal, Jacquelyn; Good, Erica; Alshaiji, Jasem; De Solo, Santiago; Ricotti, Carlos; Alvarez-Connelly, Elizabeth

    2014-01-01

    A 19-year-old girl presented with hemorrhagic acneiform lesions on the face for several months that was unresponsive to conventional acne treatment. A biopsy revealed a noninfectious suppurative granulomatous dermatitis with hemorrhage, possibly representing a ruptured folliculitis. A second biopsy revealed chronic granulomatous dermal inflammation and hemorrhage with foreign body giant cells non-infectious by stains. No vasculitis was noted in either biopsy. Later in her course she developed a severe sinusitis and eventually presented with severe fevers, rapid weight loss, sinusitis, and cough. Further workup produced the diagnosis of granulomatosis with polyangiitis (Wegener's granulomatosis). She rapidly improved with intravenous steroids and rituximab. To date, acneiform lesions have only been reported in young adult patients and may represent a clinical manifestation of granulomatosis with polyangiitis unique to this age group, as illustrated in our patient.

  20. Clinicopathological diagnosis of orofacial granulomatosis.

    Science.gov (United States)

    Afsar, Fatma Sule; Duran, Hatice Demirlendi; Yilmaz, Gungor; Ermete, Murat

    2017-01-01

    Orofacial granulomatosis is a rare chronic inflammatory disorder characterized by persistent or recurrent soft tissue swellings, oral ulceration, and other orofacial features in the absence of an identifiable granulomatous disease. We report a case of a 61-year-old woman with recurrent ulcerations and swellings in her oral mucosa. She was diagnosed as orofacial granulomatosis based upon clinicopathological correlation after exclusion of other granulomatous diseases and showed a favorable response to systemic corticosteroid treatment.

  1. Features of coexisting granulomatosis with polyangiitis in a patient with pemphigus vulgaris: a case report.

    Science.gov (United States)

    Cala, Cather M; Sami, Naveed

    2015-01-01

    Pemphigus vulgaris (PV) and granulomatosis with polyangiitis (Wegener's or GPA) are two rare autoimmune disorders. Both can involve the upper airways, and diagnosis can be difficult in the absence of extra-airway symptoms. We report the case of a patient with well-controlled PV but persistent upper respiratory tract symptoms. Further evaluation revealed perforation of the nasal septum and elevated serologies consistent with GPA. The patient improved with rituximab treatments. This case demonstrates that alternative concomitant diagnosis should be considered in patients with symptoms suggestive of recalcitrant PV and/or GPA, since these patients may require more aggressive initial treatment.

  2. Lymphomatoid granulomatosis mimicking interstitial lung disease.

    Science.gov (United States)

    Braham, Emna; Ayadi-Kaddour, Aïda; Smati, Belhassen; Ben Mrad, Sonia; Besbes, Mohammed; El Mezni, Faouzi

    2008-11-01

    Lymphoid granulomatosis is a rare form of pulmonary angiitis. This case report presents a patient with lymphoid granulomatosis in whom the clinical presentation, radiological features and the partial response to corticosteroid therapy mimicked interstitial lung disease. Lymphoid granulomatosis was only diagnosed at post-mortem examination. The range of reported clinical presentations, diagnostic approaches and outcomes are described.

  3. 韦格纳肉芽肿病合并肥厚性颅内硬脑膜炎三例并文献复习%Hyperthrophic cranial pachymeningitis in Wegener granulomatosis: 3 case report and review of liter-atures

    Institute of Scientific and Technical Information of China (English)

    吴庆军; 苏金梅; 郑文洁; 曾小峰; 张奉春; 赵岩; 唐福林

    2008-01-01

    Objective To study the clinical, laboratory, radiological and pathological findings of patients with hypertrophic cranial pachymeningitis (HCP) in Wegner's granulomatosis (WG) to improve the recognition of the disease, even when it occurs in limited form. Methods Three patients were described and English literatures of biopsy-proven pachymeningitis in WG were reviewed. Results The features of WG-associated pachymeningitis included: ① Frequently occurred early in the course of active limited WG; ② Commonly presented with sever headache and cranial neuropathies in the absence of other meningeal irritative signs; ③ Variable cerebrospinal fluid findings with mild predominantly lymphocytic pleocytosis and elevated protein concentration were major laboratorg findings; ④Elevated ESR and positive serum anti-neutrophilic cytoplasmic antibody (ANCA) could be found in most patients; ⑤ Gadolinium-enhanced brain MRI is very senitive in the detection of pachymeningitis; ⑥A dural biopsy showed granulomatous necrotizing inflammation, giant cell, and evidence of vasculitis;⑦ A favorable response to standard treatment with corticosteroid, cyclophosphamide or other cytotoxic drugs could be observed. Conclusion HCP may be the initial or cardinal manifestation of the limited form of WG. Early diagnosis by ANCA, MRI and dural biopsy may facilitate diagnosis Corticosteroid and immunosupressant are the choices of treatment.%目的 分析以肥厚性颅内硬脑膜炎(HCP)为主要表现的韦格纳肉芽肿病(WG)的临床表现、磁共振成像(MRI)和硬脑膜病理特征.方法 报道3例WG合并HCP,并复习国外文献.结果 HCP多见于局限型WG,常在发病半年内出现,表现为头痛和颅神经病,伴有呼吸道/眶部受累.半数以上患者抗中性粒细胞胞质抗体(ANCA)阳性.脑脊液改变示约1/3有轻度白细胞数增高和2/3有蛋白增高.MRI均显示硬脑膜增厚并强化.硬脑膜活检病理显示肉芽肿炎症(100%)、组织坏死(94

  4. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis.

    Science.gov (United States)

    Choi, Hyun Ah; Lee, Mi Ji; Chung, Chin-Sang

    2017-04-01

    Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

  5. Pulmonary lymphomatoid granulomatosis in a 4-year-old girl

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Subin; Kang, Mi-Jin; Lee, Jihae [Inje University Sanggye Paik Hospital, Department of Radiology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyun-Jung [Inje University Sanggye Paik Hospital, Department of Pathology, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of); Kim, Hyo-Bin [Inje University Sanggye Paik Hospital, Department of Pediatrics, 1342, Dongilro, Nowon-gu, Seoul (Korea, Republic of)

    2015-07-15

    Lymphomatoid granulomatosis is a rare lymphoproliferative disease associated with the Epstein-Barr virus that commonly affects the lung. There is limited literature on cases of pediatric lymphomatoid granulomatosis. Half of all cases of lymphomatoid granulomatosis develop during the treatment of leukemia. Herein, we describe a case of lymphomatoid granulomatosis in a previously healthy child without leukemia. (orig.)

  6. Lymphomatoid granulomatosis: two cases with skin involvement.

    Science.gov (United States)

    Camisa, C

    1989-04-01

    Lymphomatoid granulomatosis is a systemic disease marked by a polymorphous cellular infiltrate that is both angiocentric and angiodestructive. The predominant organs of involvement are lungs, skin, central nervous system, and kidneys. I describe two cases of lymphomatoid granulomatosis in association with cutaneous manifestations, stressing to the dermatologist the importance of early recognition and diagnosis of this entity.

  7. The relation between Staphylococcus aureus and Wegener's granulomatosis : Current knowledge and future directions

    NARCIS (Netherlands)

    Popa, ER; Tervaert, JWC

    2003-01-01

    To date, in the investigation of the role of S. aureus in WG, we face a paradoxical situation. On the one hand, clinical results obtained from treatment of WG patients with co-trimoxazole and studies assessing the impact of S. aureus on disease relapses strongly suggest that this bacterium contribut

  8. T-CELL REACTIVITY TO PROTEINASE-3 AND MYELOPEROXIDASE IN PATIENTS WITH WEGENERS GRANULOMATOSIS (WG)

    NARCIS (Netherlands)

    BROUWER, E; STEGEMAN, CA; HUITEMA, MG; LIMBURG, PC; KALLENBERG, CGM

    1994-01-01

    T cell-mediated immunity is hypothesized to play an important role in the pathogenesis of granulomatous inflammation and vasculitis as found in patients with WG. The antigenic specificities of those T cells remain, however, unknown. Anti-neutrophil cytoplasmic antibodies (ANCA) present in patients w

  9. Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener's)

    DEFF Research Database (Denmark)

    Martinez Del Pero, Marcos; Rasmussen, Niels; Chaudhry, Afzal;

    2013-01-01

    disease cf. 59/131 and 52/131, respectively, in remission). Nasal crusting was the most common nasal complaint recorded (52/144, 36 %) and bloody rhinorrhoea was the most common symptom in patients with disease activity. Rhinoscopy was highly sensitive in diagnosing disease activity (100 %). Subglottic...... stenosis was the most common head and neck manifestation (27/121, 22 %) and 74 % were symptomatic. In conclusion, the pattern and frequency of clinical ENT manifestations in GPA have been described in a large patient cohort. The use of tools readily available in the ENT clinic was essential to assess...

  10. Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations

    Directory of Open Access Journals (Sweden)

    Karolina Kędzierska

    2016-03-01

    Full Text Available Background: Pyoderma gangrenosum (PG is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG. The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment. Methods: Systematic review of the literature in PubMed using the search terms “Wegener granulomatosis AND Pyoderma gangrenosum” and case report. Results: The finding of 22 reports in the literature (PubMed suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide. Discussion: The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.

  11. Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Basheer Tashtoush

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis (WG, is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

  12. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Science.gov (United States)

    Ali, Robert; Baldeo, Candice; Onyenekwe, Jesse; Lala, Roshan; Landa, Cristian; Siddiqi, Anwer

    2015-01-01

    Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched. PMID:26347844

  13. Granulomatosis with polyangiitis in Tunisia

    Directory of Open Access Journals (Sweden)

    I. Ben Ghorbel

    2017-05-01

    Full Text Available Granulomatosis with polyangiitis (GPA is more frequent in Northern rather than Southern countries. Very few studies have been conducted in Africa. We have performed a retrospective descriptive study including clinical and laboratory profiles of 30 Tunisian GPA patients seen at the department of Internal Medicine of the University Hospital of la Rabta from 2000 to 2014. Mean age at initial GPA diagnosis was 46±12 years, and the average number of months between the onset of symptoms and diagnosis was 25. Seventeen (56% were male, and 13 (44% were female. Ear/nose/throat involvement occurred in 83%. Lung and renal involvement were observed in respectively 70% and 56% followed by mucocutaneous (50%, neurological (50%, ocular (33%, vascular (20%, ureteral (16%, and cardiac involvement in 10%. Cytoplasmic pattern-antineutrophil cytoplasmic antibodies (ANCA was detected in 27 (90% patients. Induction therapy consisted of intravenous cyclophosphamide pulses in 27 patients (90% and oral methotrexate in 3 patients (10%. Trimethoprime-sulfamethoxazole was used in 26 patients (86%. Maintenance therapy consisted of azathioprine in 17 cases and methotrexate in 13 cases. Relapses occurred in 36%. Eighteen patients had favorable outcome and 12 died. Our patients had a distinct phenotype with high prevalence of pleural involvement, lymph node enlargement, sensorimotor neuropathy and ureter stenosis. ENT symptoms were less frequent as inaugural presentation. Overall 2-year survival was 60%.

  14. Granulomatosis with Polyangiitis (GPA) Mimicking Tuberculosis.

    Science.gov (United States)

    Haridas, Vikram; Haridas, Kiran

    2017-03-01

    Granulomatosis with Polyangiitis (GPA) is a rare disease with varied clinical manifestations. We present a case of GPA which manifested initially with symptoms suggestive of meningeal tuberculosis. High index of suspicion and collective review of all clinical features helped in the correct diagnosis. Treatment of this case with rituximab provided significant symptomatic relief. © Journal of the Association of Physicians of India 2011.

  15. Characteristics of patients with orofacial granulomatosis.

    LENUS (Irish Health Repository)

    McCartan, B E

    2011-10-01

    Orofacial granulomatosis has mostly been described in reports of very small numbers of cases. Few large case groups have been described. The aim of this study was to describe the demographics, symptoms, clinical features and laboratory findings in a large cohort of cases.

  16. Histopathological features predictive of a clinical diagnosis of ophthalmic granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Isa, Hazlita; Lightman, Sue; Luthert, Philip J; Rose, Geoffrey E; Verity, David H; Taylor, Simon R J

    2012-01-01

    The limited form of Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's Granulomatosis (WG) primarily involves the head and neck region, including the orbit, but is often a diagnostic challenge, particularly as it commonly lacks positive anti-neutrophil cytoplasm antibody (ANCA) titres or classical features on diagnostic orbital biopsies. The purpose of this study was to relate biopsy findings with clinical outcome and to determine which histopathological features are predictive of a clinical diagnosis of GPA. Retrospective case series of 234 patients identified from the database of the UCL Institute of Ophthalmology Department of Eye Pathology as having had orbital biopsies of orbital inflammatory disorders performed between 1988 and 2009. Clinical records were obtained for the patients and analysed to see whether patients had GPA or not, according to a standard set of diagnostic criteria (excluding any histopathological findings). Biopsy features were then correlated with the clinical diagnosis in univariate and multivariate analyses to determine factors predictive of GPA. Of the 234 patients, 36 were diagnosed with GPA and 198 with other orbital pathologies. The majority of biopsies were from orbital masses (47%). Histology showed a range of acute and chronic inflammatory pictures in all biopsies, but the presence of neutrophils (PGPA. In a multivariate analysis, only tissue neutrophils (OR=3.6, P=0.01) and vasculitis (OR=2.6, P=0.02) were independently associated with GPA, in contrast to previous reports associating eosinophils and necrosis with the diagnosis. Neutrophil, eosinophil and macrophage infiltration of orbital tissues, together with vasculitis and necrosis, are all associated with a clinical diagnosis of GPA, but only neutrophil infiltration and vasculitis are independently associated with this diagnosis. These features may assist in the establishing the diagnosis of limited GPA among patients with early orbital disease, particularly

  17. Postirradiation pulmonary fibrosis complicated by aspergilloma and bronchocentric granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Makker, H.; McConnochie, K.; Gibbs, A.R. (Univ. of Wales College of Medicine, Llandough Hospital, Penarth (UK))

    1989-08-01

    An asthmatic patient requiring cortico-steroid treatment developed a pulmonary aspergilloma in an area of postmastectomy radiation fibrosis. At necropsy bronchocentric granulomatosis was also found. (author).

  18. Orofacial granulomatosis: clinical signs of different pathologies.

    Science.gov (United States)

    Troiano, Giuseppe; Dioguardi, Mario; Giannatempo, Giovanni; Laino, Luigi; Testa, Nunzio Francesco; Cocchi, Roberto; De Lillo, Alfredo; Lo Muzio, Lorenzo

    2015-01-01

    Orofacial granulomatosis (OFG) is an uncommon disease characterized by persistent or recurrent soft tissue enlargement, oral ulceration and a variety of other orofacial features. It could be an oral manifestation of a systemic disease. For a correct differential diagnosis, local and systemic conditions characterized by granulomatous inflammation should be excluded using appropriate clinical and laboratory investigations. In fact, the diagnosis of OFG may be confirmed only by histopathological identification of noncaseating granulomas. The literature from 1943 to 2014 was reviewed with emphasis on the etiology of OFG and on clinical manifestations of systemic pathologies associated with OFG. The precise cause of OFG is still unknown, although several theories have been suggested, such as infection, hereditary factors and allergy. OFG is a disease that has a wide spectrum of presentation, which may include the oral manifestation of a systemic condition such as Crohn's disease, sarcoidosis, granulomatosis with polyangiitis and Melkersson-Rosenthal syndrome.

  19. Treatment of Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    G. Valesini

    2011-09-01

    Full Text Available Treatment of Wegener’s granulomatosis, often a life-threatening disease, has greatly improved, considering that before corticosteroids and immunosuppressives were available, the average survival time of patients amounted to no more than 5 months. The management of Wegener’s granulomatosis can be divided in two stages: induction of remission and maintenance of remission. The standard regimen for the induction of remission consists of cyclophosphamide, 2 mg/kg/day orally, in combination with prednisone, 1mg/Kg/day orally, with a gradual tapering once remission has been obtained. To lower the overall cumulative dose, monthly intravenous pulses of cyclophosphamide have been evaluated. Other alternative treatments as high doses of corticosteroids, methotrexate, or plasmapheresis have been proposed, together as prophylaxis with trimethoprim-sulfamethoxazole. To minimize toxicity, for maintenance therapy other drugs are also used such as methotrexate, azathioprine, cyclosporine. Frequent therapeutic changes are needed due to the great variability of the disease; while important aspects are the recognition and treatment of relapse, and include not only the management of resistant disease, but also some particular aspects such as disease in chronic dialysis, renal transplant, pregnancy. Other cytotoxic drugs like leflunomide or mycofenolic mofetil appear to be promising, while new efforts to identify more effective and less toxic therapies include biologic products, such as high-dose immunoglobulin, TNF antagonists and other monoclonal antibodies. Many different kind of clinical trials are going on to better evaluate the real efficacy and safety of these treatments in Wegener’s granulomatosis.

  20. Orofacial granulomatosis in children: think about Crohn's disease.

    Science.gov (United States)

    Lazzerini, Marzia; Martelossi, Stefano; Cont, Gabriele; Bersanini, Chiara; Ventura, Giovanna; Fontana, Massimo; Zuin, Giovanna; Ventura, Alessandro; Taddio, Andrea

    2015-04-01

    The term orofacial granulomatosis is conventionally used to describe patients with granulomatous lesions affecting the orofacial tissues, in absence of intestinal lesions. Lip swelling and facial swelling are the most common clinical signs. Despite the fact that histologically it is not distinguishable from Crohn's disease, and that both diseases have a chronic/recurrent course, the relationship between orofacial granulomatosis and Crohn's disease is still debated. Herein we present five cases of orofacial granulomatosis. All patients presented concomitant Crohn's disease, supporting the hypothesis that orofacial granulomatosis and Crohn's disease may be one single disease. Thalidomide was effective in inducing remission of oral and intestinal symptoms in all five cases and could be considered a valid treatment opportunity for these patients. Orofacial granulomatosis and Crohn's disease may be part of the same disease; both may respond to thalidomide. Copyright © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  1. WEGENER: World Earthquake GEodesy Network for Environmental Hazard Research

    Science.gov (United States)

    Ozener, Haluk; Zerbini, Susanna; Bastos, Luisa; Becker, Matthias; Meghraoui, Mustapha; Reilinger, Robert

    2013-04-01

    WEGENER is originally the acronym for Working Group of European Geoscientists for the Establishment of Networks for Earth-science Research. It was founded in March 1981 in response to an appeal delivered at the Journées Luxembourgeoises de Geodynamique in December 1980 to respond with a coordinated European proposal to a NASA Announcement of Opportunity inviting participation in the Crustal Dynamics and Earthquake Research Program. WEGENER, during the past 32 years, has always kept a close contact with the Agencies and Institutions responsible for the development and maintenance of the global space geodetic networks with the aim to make them aware of the scientific needs and outcomes of the project which might have an influence on the general science policy trends. WEGENER was serving as Inter-commission Project 3.2, between Commission 1 and Commission 3, of the International Association of Geodesy (IAG) until 2012. Since then, WEGENER project has became the Sub-commission 3.5 of IAG commission 3, namely Tectonics and Earthquake Geodesy. In this study, we briefly review the accomplishments of WEGENER as originally conceived and outline and justify the new focus of the WEGENER consortium. The remarkable and rapid evolution of the present state of global geodetic monitoring in regard to the precision of positioning capabilities (and hence deformation) and global coverage, the development of InSAR for monitoring strain with unprecedented spatial resolution, and continuing and planned data from highly precise satellite gravity and altimetry missions, encourage us to shift principal attention from mainly monitoring capabilities by a combination of space and terrestrial geodetic techniques to applying existing observational methodologies to the critical geophysical phenomena that threaten our planet and society. Our new focus includes developing an improved physical basis to mitigate earthquake, tsunami, and volcanic risks, and the effects of natural and anthropogenic

  2. Orofacial granulomatosis in a patient with Crohn's disease.

    NARCIS (Netherlands)

    Scheur, van de M.R.; Waal, van der RI; Volker - Dieben, H.J.M.; Knol, E.C.; Starink, T.M.; Waal, van der I.

    2003-01-01

    Orofacial granulomatosis encompasses the previously recognized clinical entities Melkersson-Rosenthal syndrome and cheilitis granulomatosa. We report the case of a 39-year-old patient with cheilitis granulomatosa, intestinal Crohn's disease, and optic neuropathy. Cheilitis granulomatosa and optic ne

  3. Isolated neurological involvement of lymphomatoid granulomatosis

    Institute of Scientific and Technical Information of China (English)

    HU Yu-hua; SHAO En-de; WU Jian-liang; MENG Xian-bing

    2010-01-01

    @@ Lymphomatoid granulomatosis (LG) is a rare EpsteinBarr virus (EBV)-associated systemic granulomatous disease that is characterized by an angiocentric and occasionally angiodestructive polymorphic cellular infiltrate. It most frequently affects the lungs, kidneys,and brain.1,2 Usually the lesions are multi-systemic and multifocal. Central nervous system (CNS) involvement occurs in approximately 30% of affected patients, and patients may present with nonspecific neurological symptoms.3 Although CNS lesions usually appear as a secondary metastasis of pulmonary lesions, in rare conditions, it may be the initial or only manifestation of the disease. In this report, we describe two cases presenting with unifocal mass in the brain, histologically confirmed to be LG,

  4. Pediatric Vasculitis Initiative

    Science.gov (United States)

    2016-08-19

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

  5. Miescher's granulomatosis (granulomatosis disciformis chronica et progressiva in a non-diabetic patient – case report

    Directory of Open Access Journals (Sweden)

    Brzezińska-Wcisło Ligia

    2009-08-01

    Full Text Available Abstract Introduction Necrobiosis lipoidica diabeticorum is a rare disease of unclear etiology, that occurs in about 1% of diabetic patients. Case report We present case of granulomatosis disciformis chronica et progressiva Miescher with good response to systemic corticosteroids therapy. Patient 45 years old woman, with primary yellow-brown areas skin lesions, with foci well separated from surroundings on both lower legs, that occurred 5 years ago. In laboratory tests there was no abnormalities. Because of advance suggestion (after last admit in dermatological ward of observation according to xantogranuloma necrobioticum tests for paraproteinemia were made. Immunoelectrophoresis, IgG, IgM, IgA levels, kappa light chain, lambda heavy chain; were correct, Bence-Johns protein-negative. During hospitalization in Clinic methylprednisolone in dose of 32 mg od, vascular drugs and local steroidotherapy was applied with good therapeutic response. Conclusion We described case of typical clinical and histological characters of necrobiosis lipoidica. without diabetes-granulomatosis disciformis chronica et progressiva Miescher that despite of suspicion of proper diagnosis for a long time was not treat effective.

  6. A case of fever of unknown origin: necrotizing sarcoid granulomatosis.

    Science.gov (United States)

    Unlü, G; Onyılmaz, T A; Barış, S A; Turhan, N; Vural, C; Başyiğit, I; Boyacı, H

    2014-01-01

    Necrotizing sarcoid granulomatosis is a rare type of vasculitis; its etiology and pathogenesis are still unknown. The disease primarily affects the lungs, although extra-pulmonary involvement has been reported. The typical symptoms are cough, chest pain, dyspnea, and weight loss; high temperatures have been reported in rare cases. We present the case of a 65-year-old woman who was diagnosed with lymph node tuberculosis, for which she received treatment for six months. The patient experienced no improvement in her symptoms, which included fever, weakness and dyspnea. A re-evaluation of previously collected thoracoscopic biopsy material revealed compatibility with necrotizing sarcoid granulomatosis.

  7. Naar meer veiligheid op gebiedsontsluitingswegen : aanbevelingen voor wegvakken van de huidige 50- en 80km/uur-wegen.

    NARCIS (Netherlands)

    Dijkstra, A. & Schermers, G.

    2014-01-01

    Gebiedsontsluitingswegen — de huidige 50- en 80km/uur-wegen — behoren nog steeds tot de gevaarlijkste wegen in Nederland. De ontwerprichtlijnen voor deze wegen worden sterk verschillend toegepast. Een kwantitatieve onderbouwing van de richtlijnen en een beter georganiseerd ontwerpproces kan duidelij

  8. Eosinophilic granulomatosis with polyangiitis: an overview

    Directory of Open Access Journals (Sweden)

    Andrea eGioffredi

    2014-11-01

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

  9. [Spontaneous hemothorax revealing Wegener's vasculitis in a pregnant woman].

    Science.gov (United States)

    Serhane, Hind; Yassine, Msougar; Amro, Lamyae

    2016-01-01

    Spontaneous hemothorax is a rare condition. Its causes are multiple but sometimes they remain unknown. In some patients, thoracotomy may be the only means to determine hemothorax origin. Vasculitis have not been reported as a common cause of spontaneous hemothorax. Pregnancy does not appear to have causal or aggravating effect on spontaneous hemothorax or on vasculitis. We here report the peculiar case of a young patient presenting during pregnancy with spontaneous hemothorax secondary to Wegener's vasculitis. The latter was diagnosed by pleural biopsy performed during exploratory thoracotomy and confirmed by ANCA assays.

  10. Orbital lymphomatoid granulomatosis - a rare cause of proptosis

    Energy Technology Data Exchange (ETDEWEB)

    Du Toit, Jacqueline; Kilborn, Tracy [Department of Radiology, Red Cross Children' s Hospital, Rondebosch (South Africa); Eyssen, Ann van [Department of Oncology, Red Cross Children' s Hospital, Rondebosch (South Africa); Pillay, Komala [Department of Pathology, Red Cross Children' s Hospital, Rondebosch (South Africa)

    2015-07-15

    A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis. (orig.)

  11. "Wegener’s granulomatosis in a patient with Rheumatoid arthritis "

    Directory of Open Access Journals (Sweden)

    "Ahmadi Nejad Z

    2001-06-01

    Full Text Available Wegener’s Granulomatosis is a necrotizing granulomatous vasculitis involving small and medium sized vessels. The syndrome is classically defined as having involvement of kidney, lungs and upper respiratory tract (i.e. sinuses. Wegener’s Granulomatosis may be present in other autoimmune or inflammatory diseases, particulary systemic lupus erythematosis (SLE, but most frequently has been associated with polyarteritis and glumerulonephritis.We present a case of wegener’s Granulomatosis (WG in a middle age lady with Rheumatiod Arthritis (RA; and discuss the implications of these two conditions co-existing in one patient. As for as we are aware, through medline and interent research, this is probably the fourth case with such as association of WG and RA and the fist one in Iran.Presentation of new uncontrollable sing and symptoms, in a previously well controlled RA patient, might suggest a new overlapping syndrome like Wegener’s Granulomatosis besides to flare up of previous disease as differential diagnosis

  12. Patch testing for food-associated allergies in orofacial granulomatosis.

    LENUS (Irish Health Repository)

    Fitzpatrick, Laura

    2011-01-01

    Food-associated allergies, especially to benzoates and cinnamon-related compounds, have been associated with orofacial granulomatosis and both standard and urticarial patch testing have been used to detect such allergies. Elimination diets have also been shown to be effective in some patients.

  13. Uncommon inflammatory swelling of the lips: orofacial granulomatosis.

    Science.gov (United States)

    Hafiz, Abdul; Mufeed, Abdulla; Kandasamy, Gopinath; Krishnapillai, Rekha

    2016-01-12

    Orofacial granulomatosis (OFG) is an unusual condition associated with permanent or recurrent swelling of orofacial tissues together with oral mucosal ulceration and a variety of orofacial characteristics. The chronic inflammation inherent to OFG often displays granulomas in the subepithelial stroma. We present a case of OFG and its management. The patient responded to intralesional injections of corticosteroids.

  14. 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

  15. A Case of Wegener’s Granulomatosis Presenting with Unilateral Facial Nerve Palsy

    Directory of Open Access Journals (Sweden)

    Roy Ujjawal

    2016-01-01

    Full Text Available Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.

  16. Esclerite nodular e poliangiite granulomatosa (Wegener mimetizando tuberculose

    Directory of Open Access Journals (Sweden)

    Cybelle Moreno Luize Franco

    2015-04-01

    Full Text Available Poliangiite granulomatosa é uma doença sistêmica que afeta qualquer órgão, com predileção pelo trato respiratório superior, pulmões e rins. Este artigo tem como objetivo relatar um caso atípico de uma paciente com esclerite nodular como manifestação inicial da poliangiite granulomatosa (Wegener, mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuía nódulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos álcool-ácido resistentes (BAAR. O diagnóstico histológico revelou uma vasculite granulomatosa sugestiva de vasculite não infecciosa (poliangiite granulomatosa. Foi iniciada pulsoterapia com ciclofosfamida.

  17. Wegener's thinking about the mechanism: Greenland and Iceland

    Science.gov (United States)

    Jacoby, Wolfgang

    2015-04-01

    Wegener's early Arctic expeditions to Greenland (1906-08 and 1912-13 with a stop in Iceland) suggest a significant affect on his thinking about the mechanism of continental drift till his death in 1930. Beside his specialized work in meteorology and the Arctic, he had a broad general interest in science especially of the earth system as a whole. The drift idea occurred to him in 1910 on the basis of new data on geomorphology (Atlantic seafloor), supported by geophysics, geology and palaeontology. In his 1912 initial public talk and ensuing paper he mentioned something akin to seafloor spreading and refuted the continental relict hypothesis from break-up for the mid-Atlantic ridge. But 1912 he bypassed the tension fractures in Iceland and in Greenland (1912-13) he experienced the rheology of ice, brittle and viscous, when thinking about the drift of SIAL continents through the SIMA mantle (as documented in his diaries). When in 1915 rewriting his 1012 paper as the book "Die Entstehung der Kontinente und Ozeane" he had given up the early idea for that of floating continental rafts. It is tempting to speculate why. Rheology of rocks was clearly described. But some misconceptions distracted him from the correct relationships: (1) Data of the time suggested that sialic rock is more solid than mafic rock which would soften at lower temperature (contrary to present knowledge) and (2) convection in the atmosphere, well known to him, seemed to be no model for convection in the mantle, although rafting continents implied mantle flow. Did the rheologies appear too different to him? Not before the mid twenties (as documented in the 4th edition of his book, 1929) did Wegener admit that mantle convection might be the answer. A great spirit was misled but clearly saw that the phenomenon of drift, based on observations, is not refuted by the lack of an explanation.

  18. Idiopathic orofacial granulomatosis in a young patient: A rare entity

    Directory of Open Access Journals (Sweden)

    Gurumoorthy Kaarthikeyan

    2012-01-01

    Full Text Available The granular enlargement of the gingiva may be the first clinical manifestation of Orofacial granulomatosis, preceding other local or systemic manifestations. The term Idiopathic Orofacial granulomatosis (OFG refers to conditions restricted to the oral region without any identifiable systemic granulomatous diseases. We report a case of nine year old female patient with gingival enlargement as the sole manifestation without any systemic involvement. Laboratory investigations were done to rule out tuberculosis, sarcoidosis, allergic hypersensitivity, Crohn′s disease. The patient was treated by gingivectomy and there is complete remission of the lesion without any recurrence. Thus the early investigations and diagnosis of OFG can help in the prevention of future systemic complications.

  19. A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

    Directory of Open Access Journals (Sweden)

    Ghulam Rehman Mohyuddin

    2012-01-01

    Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

  20. Wegener′s granulomatosis disease mimicking pulmonary tuberculosis

    Directory of Open Access Journals (Sweden)

    Naveen Pandhi

    2015-01-01

    Full Text Available Wegener′s granulomatosis (WG is an uncommon autoimmune disease with multi-system involvement that manifests as vasculitis, granulomatosis, and necrosis. While its standard form involves the upper and lower respiratory tracts and kidneys, it may essentially involve any organ. We present a case in a young female patient presenting with symptoms of cough with expectoration, fever, dyspnea and chest discomfort, having cavitating lesion in right upper lobe and nodule in left upper lobe with bilateral paranasal sinus involvement on computed tomography. Allergic rhino-sinusitis with pulmonary tuberculosis was suspected, and patient was investigated further. Upon further investigation, renal involvement was detected, and serology revealed cytoplasmic antineutrophil cytoplasmic antibody. WG was suspected and was proven by histopathology of nasal tissue which revealed necrotizing granulomas.

  1. Granulomatosis with Polyangiitis Presenting as a Parotid Gland Abscess

    Directory of Open Access Journals (Sweden)

    Blenda Dias

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA is a small-vessel vasculitis consisting of necrotizing granulomatous lesions in airways and focal necrotizing glomerulonephritis. However, it may affect other sites such as the skin, central nervous system, eyes, heart, gastrointestinal tract, and liver. We describe a rare case of GPA in which the initial manifestation was the involvement of the parotid gland mimicking a pyogenic abscess.

  2. Archive of Geosample Information from the Alfred-Wegener-Institut (AWI) Core Repository

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Alfred-Wegener-Institut (AWI) made a one-time contribution to the Index to Marine and Lacustrine Geological Samples (IMLGS) database of metadata describing 1820...

  3. Computed tomographic characteristics of eosinophilic pulmonary granulomatosis in five dogs.

    Science.gov (United States)

    Fina, Caroline; Vignoli, Massimo; Terragni, Rossella; Rossi, Federica; Wisner, Erik; Saunders, Jimmy H

    2014-01-01

    Canine pulmonary eosinophilic granulomatosis is a rare inflammatory pulmonary disease characterized by formation of eosinophilic granulomas that tend to obliterate the normal pulmonary architecture. The purpose of this retrospective study was to describe the CT characteristics of confirmed idiopathic pulmonary eosinophilic granulomatosis in a group of dogs. Five dogs met inclusion criteria. All patients were young adult dogs of variable breeds. No dog had concurrent occult heartworm disease. Computed tomographic characteristics most commonly included pulmonary masses and nodules of variable size, and lesions were most commonly located in the caudal lung lobes. Four dogs had large pulmonary masses with or without additional nodules and one dog had nodular lesions disseminated throughout the entire lung parenchyma. All large eosinophilic granulomas were smoothly margined, heterogeneous pulmonary masses displaying heterogeneous contrast enhancement. A honeycomb-like enhancement pattern was observed in all but one mass and consisted of multiple hyperattenuating rims delineating central hypoattenuating areas, suggestive of bronchiectatic lung with peripheral enhancing airway walls and fluid-filled, necrotic bronchial lumen. One dog had evidence of tracheobronchial lymphadenopathy. Findings indicated that canine eosinophilic pulmonary granulomatosis should be included as a differential diagnosis for dogs with CT characteristics of multiple pulmonary masses and/or nodules in caudal lung lobes, and a honeycomb-like enhancement pattern in masses after intravenous administration of iodinated contrast medium.

  4. Occult Renal Granulomatous Inflammatory Lesions in Granulomatosis with Polyangiitis Detected by 18F-FDG PET/CT.

    Science.gov (United States)

    Fu, Zhanli; Liu, Meng; Li, Ziao; Zhang, Jin; Li, Qian

    2017-09-01

    A 33-year-old woman with a 7-month history of granulomatosis with polyangiitis F-FDG PET/CT scan due to low-grade intermittent fever and elevation of serum inflammatory markers, which revealed multiple hypermetabolic lesions in bilateral kidneys. Biopsy of a lesion showed granulomatosis with polyangiitis, consistent with renal involvement of granulomatosis with polyangiitis.

  5. Infectious complication or exacerbation of granulomatosis with polyangiitis?

    Science.gov (United States)

    Masiak, Anna; Struk-Panfill, Małgorzata; Zdrojewski, Zbigniew

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is a primary, systemic small vessel vasculitis. The respiratory tract is typically involved in the course of the disease. Abnormalities on the chest radiograph are noted in more than 70% patients at some point during their disease history. In some clinical situations it is difficult to distinguish whether symptoms result from the underlying disease or are a symptom of infection. In these clinical situations, chest computed tomography (CT) can be very useful. We present a patient with GPA localized mainly in the respiratory tract with sudden deterioration of the general state and new abnormalities revealed in the CT of the chest.

  6. Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

    Directory of Open Access Journals (Sweden)

    Diana L. Franco

    2014-10-01

    Full Text Available Eosinophilic granulomatosis with polyangiitis (EGPA, formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder.

  7. Recurrence of Intravenous Talc Granulomatosis following Single Lung Transplantation

    Directory of Open Access Journals (Sweden)

    Richard C Cook

    1998-01-01

    Full Text Available Advanced pulmonary disease is an unusual consequence of the intravenous injection of oral medications, usually developing over a period of several years. A number of patients with this condition have undergone lung transplantation for respiratory failure. However, a history of drug abuse is often considered to be a contraindication to transplantation in the context of limited donor resources. A patient with pulmonary talc granulomatosis secondary to intravenous methylphenidate injection who underwent successful lung transplantation and subsequently presented with recurrence of the underlying disease in the transplanted lung 18 months after transplantation is reported.

  8. Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy: Results From the European Renal Association-European Dialysis and Transplant Association Registry

    NARCIS (Netherlands)

    Hruskova, Z.; Stel, V.S.; Jayne, D.; Aasarod, K.; Meester, J. de; Ekstrand, A.; Eller, K.; Heaf, J.G.; Hoitsma, A.J.; Jimenez, C. Martos; Ravani, P.; Wanner, C.; Tesar, V.; Jager, K.J.

    2015-01-01

    BACKGROUND: This study describes the incidence and outcomes of European patients requiring renal replacement therapy (RRT) for kidney failure due to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). STUDY DESIGN: Cohort study. SETTING & PARTICIPANTS: 12 renal registries providi

  9. In vitro T lymphocyte responses to proteinase 3 (PR3) and linear peptides of PR3 in patients with Wegener's granulomatosis (WG)

    NARCIS (Netherlands)

    Van der Geld, YM; Huitema, MG; Franssen, CFM; Limburg, PC; Kallenberg, CGM; van der Zee, R.

    2000-01-01

    T cell-mediated immunity is thought to play an important role in the pathogenesis of WG. In previous studies a minority of WG patients as well as some healthy controls showed in vitro proliferation of their peripheral blood mononuclear cells (PBMC) to PR3, the main autoantigen in WG. The relevant pe

  10. Pulmonary angiocentric lymphoma (lymphomatoid granulomatosis) in a donkey.

    Science.gov (United States)

    du Toit, N; Genovese, L M; Dalziel, R G; Smith, S H

    2012-01-01

    A 36-year-old donkey developed dyspnoea, pyrexia, hypoalbuminaemia and oedema. Following continued clinical deterioration the donkey was humanely destroyed. Grossly, there were numerous nodules (5-10mm) scattered throughout the lung. Microscopically, the lung was infiltrated by an angiocentric and bronchocentric to diffuse mixed population of small mature and atypical lymphocytes, histiocytes, plasma cells and fewer eosinophils. The infiltrate was composed of numerous small mature and fewer atypical CD3(+) T lymphocytes. Low numbers of CD20(+) and CD79a(+) B cells, some atypical, accompanied the T cells. These infiltrates were consistent with an angiocentric lymphoma and resembled lymphomatoid granulomatosis, an Epstein-Barr virus (EBV)-associated human tumour. Immunohistochemistry for EBV latent membrane protein and polymerase chain reaction analysis for equine gamma herpesvirus DNA were negative. To the authors' knowledge this is the first case of angiocentric lymphoma reported in a donkey and the first case of lymphomatoid granulomatosis-type disease in an animal in which possible concurrent infection with a gamma herpesvirus has been investigated.

  11. /sup 67/Ga scanning in talc-induced pulmonary granulomatosis

    Energy Technology Data Exchange (ETDEWEB)

    Brown, D.G.; Aguirre, A.; Weaver, A.

    1980-04-01

    We describe a case of pulmonary granulomatosis in a user who habitually injected methylphenidate (Ritalin) intravenously; symptomatic and objective improvement occurred with corticosteroid therapy. A scan of the lungs using raioactive /sup 67/Ga showed an increased concentration of /sup 67/Ga throughout both lungs. There was a reduction in abnormal accumulation of /sup 67/Ga, improvement in the arterial oxygen pressure and the diffusing capacity for carbon monoxide, and a reduction in the infiltrate on the chest x-ray film two months after the institution of therapy. Before treatment the patient's symptoms and arterial deoxygenation increased despite the cessation of her drug abuse, thus raising the question of a self-perpetuating inflammatory process in a case of pulmonary deposition of talc.

  12. Blijvend vlakke wegen effect van variaties in de ondergrond op de voorspelling van langsvlakheid

    NARCIS (Netherlands)

    Gruijters, S.

    2009-01-01

    Dit rapport bevat de resultaten van het onderzoek dat door Deltares TNO is uitgevoerd in de periode 2007-2009 binnen het Delft Cluster onderzoek Blijvend Vlakke Wegen. Het onderzoek is gericht op het opzetten van een methodiek om variaties in de ondergrond te vertalen naar te verwachten restzettings

  13. Role of WEGENER (World Earthquake GEodesy Network for Environmental Hazard Research) in monitoring natural hazards (Invited)

    Science.gov (United States)

    Ozener, H.; Zerbini, S.; Bastos, M. L.; Becker, M. H.; Meghraoui, M.; Reilinger, R. E.

    2013-12-01

    WEGENER was originally the acronym for Working Group of European Geoscientists for the Establishment of Networks for Earth-science Research. It was founded in March 1981 in response to an appeal delivered at the Journées Luxembourgeoises de Geodynamique in December 1980 to respond with a coordinated European proposal to a NASA Announcement of Opportunity inviting participation in the Crustal Dynamics and Earthquake Research Program. WEGENER, during the past 33 years, has always kept a close contact with the Agencies and Institutions responsible for the development and maintenance of the global space geodetic networks with the aim to make them aware of the scientific needs and outcomes of the project which might have an influence on the general science policy trends. WEGENER served as Inter-commission Project 3.2, between Commission 1 and Commission 3, of the International Association of Geodesy (IAG) until 2012. Since then, WEGENER project has become the Sub-commission 3.5 of IAG commission 3, namely Tectonics and Earthquake Geodesy. In this presentation, we briefly review the accomplishments of WEGENER as originally conceived and outline and justify the new focus of the WEGENER consortium. The remarkable and rapid evolution of the present state of global geodetic monitoring in regard to the precision of positioning capabilities (and hence deformation) and global coverage, the development of InSAR for monitoring strain with unprecedented spatial resolution, and continuing and planned data from highly precise satellite gravity and altimetry missions, encourage us to shift principal attention from mainly monitoring capabilities by a combination of space and terrestrial geodetic techniques to applying existing observational methodologies to the critical geophysical phenomena that threaten our planet and society. Our new focus includes developing an improved physical basis to mitigate earthquake, tsunami, and volcanic risks, and the effects of natural and

  14. Mechanisms of Disease : pathogenesis and treatment of ANCA-associated vasculitides

    NARCIS (Netherlands)

    Kallenberg, Cees G M; Heeringa, Peter; Stegeman, Coen A

    2006-01-01

    Wegener's granulomatosis and microscopic polyangiitis are idiopathic systemic vasculitides strongly associated with antineutrophil cytoplasmic autoantibodies (ANCA). In Wegener's granulomatosis, ANCA are mostly directed against proteinase 3 (PR3), whereas in microscopic polyangiitis ANCA are directe

  15. Characterisation of a Swedish cohort with orofacial granulomatosis with or without Crohn's disease.

    Science.gov (United States)

    Gale, G; Östman, S; Rekabdar, E; Torinsson Naluai, Å; Högkil, K; Hasséus, B; Saalman, R; Jontell, M

    2015-01-01

    To compare oral manifestations in a Swedish cohort of patients with orofacial granulomatosis with or without Crohn's disease and to assess NOD2 polymorphisms in the two groups. Twenty-nine patients with orofacial granulomatosis were included. Demographics, disease history, clinical features and concurrent Crohn's disease were recorded. DNA was extracted from buccal swabs and examined for NOD2 variants Arg702Trp, Gly908Arg and Leu1007fsinsC, all previously linked to gastrointestinal Crohn's disease. Twelve of 29 patients were diagnosed with coexisting gastrointestinal Crohn's disease, and of whom 21 were males. Symptom duration was significantly longer for the orofacial granulomatosis group com-pared to the group with coexisting Crohn's disease (P orofacial granulomatosis patients also perceived their overall discomfort, aesthetic problems and social discomfort as more severe. No significant differences in the clinical presentation of oral lesions between the two groups were found. None of the patients with orofacial granulomatosis carried any of the NOD2 variations, whereas four of the 12 patients with coexisting Crohn's disease had a NOD2 variant (Arg702Trp). The two patient groups had similar phenotypic characteristics but seemed to have genotypic differences regarding NOD2. The Swedish cohort differed in their clinical characteristics from patients reported in other geographical regions. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  16. Lymphomatoid Granulomatosis: CT and FDG-PET Findings

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Jonathan H. [National Jewish Health, Denver (United States); Wu, Carol C.; Gilman, Matthew D.; Palmer, Edwin L.; Hasserjian, Robert P.; Sphepar, Jo-Anne O. [Massachusetts General Hospital, Boston (United States)

    2011-11-15

    Lymphomatoid granulomatosis (LG) is a rare, aggressive extranodal Epstein-Barr virus (EBV)-positive B-cell lymphoproliferative disease. The purpose of our study was to analyze the CT and fluorodeoxyglucose positron emission tomography (FDG-PET) findings of pulmonary LG. Between 2000 and 2009, four patients with pathologically proven pulmonary LG and chest CT were identified. Two of these patients also had FDG-PET. Imaging features of LG on CT and PET were reviewed. Pulmonary nodules or masses with peribronchovascular, subpleural, and lower lung zonal preponderance were present in all patients. Central low attenuation (4 of 4 patients), ground-glass halo (3 of 4 patients), and peripheral enhancement (4 of 4 patients) were observed in these nodules and masses. An air-bronchogram and cavitation were seen in three of four patients. FDG-PET scans demonstrated avid FDG uptake in the pulmonary nodules and masses. Pulmonary LG presents with nodules and masses with a lymphatic distribution, as would be expected for a lymphoproliferative disease. However, central low attenuation, ground-glass halo and peripheral enhancement of the nodules/masses are likely related to the angioinvasive nature of this disease. Peripheral enhancement and ground-glass halo, in particular, are valuable characteristic not previously reported that can help radiologists suggest the diagnosis of pulmonary LG.

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

    Directory of Open Access Journals (Sweden)

    Shahedi K

    2013-10-01

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

  18. Long-term outcome of lung transplantation in previous intravenous drug users with talc lung granulomatosis.

    Science.gov (United States)

    Weinkauf, J G; Puttagunta, L; Nador, R; Jackson, K; LaBranche, K; Kapasi, A; Mullen, J; Modry, D L; Stewart, K C; Thakrar, M; Doucette, K; Lien, D C

    2013-01-01

    Talc lung granulomatosis results from the intravenous use of medication intended for oral use. Talc (magnesium silicate) acts as filler in some oral medications; when injected intravenously, it deposits in the lungs leading to airflow obstruction and impaired gas exchange. Allocation of donor lungs to previous intravenous drug users is controversial. After a careful selection process, 19 patients with talc lung granulomatosis have received lung allografts in our program. Long-term survival for these patients is excellent and our results suggest the previous use of intravenous drugs should not necessarily preclude lung transplantation.

  19. Wegener’s granulomatosis and environmental factors in Western Montana

    Directory of Open Access Journals (Sweden)

    Andrew Samuel Zeft

    2010-12-01

    Full Text Available The objective of our study was to determine whether exposure to silica or other environmental factors is associated with developing Wegener’s granulomatosis (WG, in a geographically isolated region of Western Montana. We sought to identify and interview all cases of WG diagnosed during 1993-2006 among residents of a ten-county region of Western Montana, as well as a group of demographically similar controls (n=39 without autoimmune disease. In the interview, we ascertained occupational silica and other exposures (metals, solvents, pesticides, tobacco. We enumerated 32 cases of WG, of whom 27 were included in the case-control study. Overall, a history of silica exposure was not associated with WG (OR = 0.68, 95% CI: 0.13-3.27, although there was a suggestion of increase in risk among persons with relatively recent (OR=2.67, 95% CI: 0.54-17.2, heavy (OR=1.82, 95% CI: 0.09-112.9, and prolonged (OR=1.53, 95% CI: 0.16-20.0 exposures. A history of having worked in the mining industry was associated with WG (six cases including three with no silica exposure, zero controls, lower 95% CI: 1.53. Risk was not associated with occupational or aerial pesticide exposure, but with residential rodenticide use (OR=12.15, 95% CI: 1.54-552. Occupational exposure to metals or solvents was not associated with WG, nor was a history of cigarette smoking. Results of earlier studies of WG support the hypothesis that silica exposure adversely influences the risk of developing WG. Our small study of WG failed to identify an association with silica overall, but the results are compat­ible with an increased risk in persons with relatively heavy, prolonged, and/or recent exposure.

  20. Detailed Follow-up Study of Pediatric Orofacial Granulomatosis Patients.

    Science.gov (United States)

    Haaramo, Anu; Alapulli, Heikki; Aine, Liisa; Saarnisto, Ulla; Tuokkola, Jetta; Ruuska, Tarja; Sipponen, Taina; Pitkäranta, Anne; Kolho, Kaija-Leena

    2017-10-01

    Orofacial granulomatosis (OFG) is a chronic inflammatory condition affecting the orofacial area. Its connection to Crohn disease (CD) is debated. Our aim was to describe a cohort of pediatric patients with OFG in detail, study the long-term behavior of OFG, and evaluate factors predicting CD in patients with OFG. We invited patients diagnosed with OFG at 2 university hospitals, Finland for a follow-up appointment. Patients (n = 29) were examined by a dentist and an otorhinolaryngologist using a structural schema. Orofacial findings were also recorded using digital photographing. Patients filled in questionnaires about general health and special diets. Patients' nutrition was evaluated from food records. The findings were compared between patients with OFG only and OFG with CD. Patients with CD had more findings in the orofacial area (total score for orofacial findings median 11) compared to patients with OFG only (total score median 7.5). There was no statistically significant difference in the type of lesions between these groups, except the upper lip was more often affected in patients with CD (n = 11) than in patients with OFG only (n = 0). Most of the patients had normal otorhinolaryngological findings. All patients with elevated anti-Saccharomyces cerevisiae antibody A levels had CD (n = 6) and they presented with more orofacial findings (total score) than patients with normal levels of anti-S cerevisiae antibody A (P = 0.0311). Long-term follow-up of pediatric-onset patients with OFG shows good prognosis. Patients with OFG do not seem to have otorhinolaryngological comorbidity. Anti-S cerevisiae antibody A may serve as a factor to indicate the possible presence of underlying CD in patients with OFG, but further studies are requested.

  1. Recurrent Septic Arthritis Due to Achromobacter xylosoxidans in a Patient With Granulomatosis With Polyangiitis.

    Science.gov (United States)

    Patel, Payal K; von Keudell, Arvind; Moroder, Philipp; Appleton, Paul; Wigmore, Robin; Rodriguez, Edward K

    2015-12-01

    We report a case of recurrent Achromobacter xylosoxidans infections including bacteremia, sepsis, septic joints and endocarditis in a 72 year old female with granulomatosis with polyangiitis. Achromobacter xylosoxidans is a gram negative bacteria increasingly identified in immunocompromised patients. Surgical and medical therapy may need to be combined.

  2. Orofacial granulomatosis in children can be the initial manifestation of systemic disease: a presentation of two cases

    DEFF Research Database (Denmark)

    Simonsen, Anne Birgitte; Deleuran, Mette

    2014-01-01

    Orofacial granulomatosis is a chronic granulomatous condition characterized by relapsing and remitting lip swelling and oral involvement that may include deep ulcers, tags and cobblestone formation. It occurs as an independent entity but also in conjunction with systemic diseases such as tubercul......Orofacial granulomatosis is a chronic granulomatous condition characterized by relapsing and remitting lip swelling and oral involvement that may include deep ulcers, tags and cobblestone formation. It occurs as an independent entity but also in conjunction with systemic diseases...

  3. Voriconazole-induced periostitis causing arthralgias mimicking a flare of granulomatosis with polyangiitis.

    Science.gov (United States)

    Gladue, Heather S; Fox, David A

    2013-12-01

    We describe a case of voriconazole-induced periostitis that occurred in a 68-year-old woman with granulomatosis with polyangiitis. Our patient presented with months of severe hip pain limiting her daily activities, which was initially felt to be a flare of her granulomatosis with polyangiitis. However, upon further review, she had an elevated alkaline phosphatase and periostitis on her hip radiograph; voriconazole was held, and within 2 days she had marked improvement in her pain. Although this clinical syndrome is well documented in transplant patients, it is a rare complication in patients with autoimmune disorders. However, it is important because it may cause severe arthralgias that can mimic a flare of rheumatic diseases.

  4. Chronic meningitis with multiple cranial neuropathies: A rare initial presentation of Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Vikas Gupta

    2013-01-01

    Full Text Available Wegener′s granulomatosis (WG is a systemic necrotizing vasculitis that affects the small blood vessels. It mainly affects the upper and lower respiratory tract and kidneys. Central nervous system (CNS involvement is rare, and has been reported only in about 8% of cases during the course of illness. Initial presentation with neurologic affection, particularly chronic hypertrophic meningitis is very unusual. We report the case of a 34 year old male who presented with chronic hypertrophic meningitis and multiple cranial nerve involvement as the initial manifestation, without respiratory and renal symptoms. This case highlights the difficulties in diagnosing a rare disease with rarer presentation, and at the same time illustrates that Wegener′s granulomatosis should be considered in the differential diagnosis of chronic meningitis.

  5. Granulomatosis with Polyangiitis with Bilateral Facial Palsy and Severe Mixed Hearing Loss

    Directory of Open Access Journals (Sweden)

    Agnieszka Wawrzecka

    2016-01-01

    Full Text Available Granulomatosis with polyangiitis is autoimmune and rare disease. It affects many organs, but the most often affected organs are the nose, lungs, and kidneys. It is part of vasculitis and causes an autoimmune attack by an abnormal type of circulating antibody termed ANCAs against small blood vessels. Disease concerns both men and women with a peak age of presentation in the sixth and seven decades. Typically upper and lower respiratory tract and kidneys are involved. Otitis externa, otitis media, or mastoiditis rarely occurs in granulomatosis with polyangiitis. Deafness is the most dangerous aural complication. Histological examination of biopsy is often not specific. A case of GPA with bilateral otitis media, bilateral deafness, and bilateral facial palsy with fatal course is presented.

  6. Two cases of extrapulmonary onset granulomatosis with polyangiitis which caused diffuse alveolar haemorrhage

    Directory of Open Access Journals (Sweden)

    Halide Kaya

    2014-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA is a rare form of vasculitis. Multidisciplinary therapeutic approach and early diagnosis assume vital importance in management of patients with diffuse alveolar haemorrhage caused by GPA, which is a rare complication. The purpose of this study was to present the diagnostic and therapeutic challenges experienced by clinicians in management of two severe cases of GPA with insidious extrapulmonary manifestations which rapidly progressed into acute kidney injury, alveolar haemorrhage and acute respiratory failure.

  7. Pachymeningitis in Granulomatosis with Polyangiitis: A Case Report and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Grigorios T. Sakellariou

    2013-01-01

    Full Text Available Central nervous involvement, mainly with symptoms of cranial neuropathies, occurs in 2–8% of patients with granulomatosis with polyangiitis (GPA. Meningeal involvement, with persistent and severe headache as main manifestation and abnormal thickening and enhancement of the dural mater on postcontrast magnetic resonance imaging, is extremely rare. We present a case of pachymeningitis due to limited GPA, providing simultaneously a literature review.

  8. [C-ANCA positive necrotising scleritis and multiple sclerosis compatible with ocular Wegener: treatment with rituximab].

    Science.gov (United States)

    Aldasoro-Cáceres, V; Aldasoro-Cáceres, I; Pérez-Moreiras, J V; Murié-Fernández, M; Ibáñez-Bosch, R

    2014-01-01

    A patient diagnosed with necrotizing scleritis, c-ANCA+ an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005-2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed. Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  9. Refractory optic perineuritis due to granulomatosis with polyangiitis successfully treated with methotrexate and mycophenolate mofetil combination therapy

    Science.gov (United States)

    Kimura, Yoshitaka; Asako, Kurumi; Kikuchi, Hirotoshi; Kono, Hajime

    2017-01-01

    Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis. These were manageable only by means of weekly methotrexate and mycophenolate mofetil combination therapy but not with methotrexate, mycophenolate mofetil, intravenous cyclophosphamide, rituximab, azathioprine, or cyclosporine individually. PMID:28293459

  10. Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis

    Directory of Open Access Journals (Sweden)

    Hohenforst-Schmidt W

    2013-07-01

    Full Text Available Wolfgang Hohenforst-Schmidt,1 Arndt Petermann,2 Aikaterini Visouli,3 Paul Zarogoulidis,4 Kaid Darwiche,5 Ioanna Kougioumtzi,6 Kosmas Tsakiridis,3 Nikolaos Machairiotis,6 Markus Ketteler,2 Konstantinos Zarogoulidis,4 Johannes Brachmann11II Medical Clinic, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 2Division of Nephrology, Coburg Clinic, University of Wuerzburg, Coburg, Germany; 3Cardiothoracic Surgery Department, “Saint Luke” Private Hospital, Thessaloniki, Greece; 4Pulmonary Department, “G Papanikolaou” General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece; 5Department of Interventional Pneumology, Ruhrlandklinik, West German Lung Center, University Hospital, University of Duisburg-Essen, Essen, Germany; 6Surgery Department (National Health System, University General Hospital of Alexandroupolis, Alexandroupolis, GreeceAbstract: Extracorporeal membrane oxygenation (ECMO is increasingly applied in adults with acute refractory respiratory failure that is deemed reversible. Bleeding is the most frequent complication during ECMO support. Severe pre-existing bleeding has been considered a contraindication to ECMO application. Nevertheless, there are cases of successful ECMO application in patients with multiple trauma and hemorrhagic shock or head trauma and intracranial hemorrhage. ECMO has proved to be life-saving in several cases of life-threatening respiratory failure associated with pulmonary hemorrhage of various causes, including granulomatosis with polyangiitis (Wegener’s disease. We successfully applied ECMO in a 65-year-old woman with acute life-threatening respiratory failure due to diffuse massive pulmonary hemorrhage secondary to granulomatosis with polyangiitis, manifested as severe pulmonary-renal syndrome. ECMO sustained life and allowed disease control, together with plasmapheresis, cyclophosphamide, corticoids, and renal replacement therapy. The patient was successfully weaned from ECMO

  11. Pulmonary sarcoid-like granulomatosis induced by aluminum dust: report of a case and literature review

    Institute of Scientific and Technical Information of China (English)

    CAI Hou-rong; CAO Min; MENG Fan-qing; WEI Jing-yi

    2007-01-01

    @@ Awide range of pulmonary pathology is attributed to aluminum dust exposure, including interstitial fibrosis,1-5 desquamative interstitial pneumonia,6 and pulmonary alveolar proteinosis.7 To our knowledge,granulomatous lung disease induced by aluminum dust is rare, only two cases were reported in literatures.8,9 We had the opportunity to make a diagnosis of pulmonary sarcoid-like granulomatosis in a patient exposed to aluminum dust. Herein, we report the clinical history,radiographic and histopathological findings in this patient,and the detailed mineralogical analyses performed on lung tissue obtained by open lung biopsy.

  12. Pulmonary lymphomatoid granulomatosis: Report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    D B Olusina

    2011-01-01

    Full Text Available Lymphomatoid granulomatosis (LYG is a rare angiocentric lymphoproliferative process predominantly affecting the lung. The diagnosis of this condition is often difficult as the physical signs, history, chest x-ray, and routine laboratory investigations are usually non-specific. Nevertheless, it is important to establish a tissue diagnosis, as this lymphoproliferative disorder can be refractory to treatment and even progress to overt lymphoma. We report a case of pulmonary LYG in a 52-year old Nigerian man of Ibo extraction treated in our centre in 2001 and followed up for a year. The difficulty in making diagnosis is highlighted and treatment modality discussed.

  13. A Rare Manifestation of Crohn's Disease: Sinonasal Granulomatosis. Report of a Case and Review of Literature

    Directory of Open Access Journals (Sweden)

    Lilia Baili

    2014-10-01

    Full Text Available Crohn’s disease is a granulomatous inflammatory bowel disease. Its pathologic findings include non-contiguous chronic inflammation and non-caseating granulomas, sometimes with extra-intestinal localizations. Sinonasal manifestations of Crohn’s disease are quite rare and only a few cases have been reported up to date in the worldwide literature. They are characterized by chronic mucosal inflammation, obstruction, bleeding and occasionally septal perforation. We report a case of sinonasal granulomatosis revealing Crohn’s disease in a 22-year-old woman and go over the available literature on sinonasal involvement in Crohn’s disease.

  14. A rare case of primary orofacial granulomatosis of gingiva during pregnancy

    Directory of Open Access Journals (Sweden)

    Vaibhavi Joshipura

    2015-01-01

    Full Text Available Orofacial granulomatosis (OFG comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

  15. A rare case of primary orofacial granulomatosis of gingiva during pregnancy.

    Science.gov (United States)

    Joshipura, Vaibhavi; Mahantesha, S; Subbaiah, Shobha Krishna; Lakkasetty, Yogesh T

    2015-01-01

    Orofacial granulomatosis (OFG) comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

  16. Wegener′s granulomatosis presenting as spontaneous pneumothorax in young adult

    Directory of Open Access Journals (Sweden)

    Sunil Kumar

    2014-01-01

    Full Text Available Pulmonary involvement in Wegener′s granulomatosis (WG usually starts with nonspecific symptoms such as cough, dyspnea, hemoptysis, and pleuritis. Spontaneous pneumothorax as initial presentation is extremely rare. Although its real incidence is unknown, according to different classic series, it ranges between 3 and 5% of the cases. In this case, a 28-year-old male presented with complaints of epistaxis and breathlessness, which was diagnosed as WG with pneumothorax on the basis of chest X-ray and computed tomography (CT chest and pathological confirmation by high level of serum cytoplasmic antineutrophil cytoplasmic antibody (ANCA.

  17. CT引导经皮肺切割活检术诊断活动性韦格纳肉芽肿%Percutaneous CT -guided Core Needle Biopsy of Lungs in the Diagnosis of Active Wegener's Granulomatosis

    Institute of Scientific and Technical Information of China (English)

    陈敏; 郭佑民; 张少娟; 杨健; 牛刚; 汪军峰

    2005-01-01

    目的探讨CT引导下经皮肺芯针切割活检术(percutaneous CT-guided core needle biopsy,PTCB)对活动性韦格纳肉芽肿(WG)的诊断价值以及指导临床治疗的意义.方法8例经PTCB行病理组织学检查WG病人,根据其临床资料作BVAS评分,其结果与病理对照研究.结果8例病人经PTCB取得足够的组织学标本,病理结果:①坏死性血管炎(75%),②肉芽性炎症(62.5%),③实质坏死(50%),④肺出血改变(12.5%),⑤纤维化(12.5%).确诊时BVAS平均分数为10分,所有病人确诊后都进行了糖皮质激素联合细胞毒药物冲击治疗,临床有不同程度好转,治疗4周后平均分数为2.5分.结论多部位、多次经皮肺切割活检可以准确的诊断活动性WG,指导临床早期治疗.

  18. Successful endonasal dacryocystorhinostomy in a patient with Wegener’s granulomatosis

    Directory of Open Access Journals (Sweden)

    P Eloy

    2009-11-01

    Full Text Available P Eloy, E Leruth, B Bertrand, Ph RombauxENT and HNS department, Cliniques Universitaires de Mont-Godinne, Université Catholique de Louvain, 5530, Yvoir, BelgiumAbstract: Wegener’s granulomatosis (WG is one form of idiopathic autoimmune vasculitis. The disease has a predilection for the upper and lower respiratory tracts (lungs, nose, sinus, and kidneys. WG may be systemic, severe, and potentially lethal, but it may also be limited to the otolaryngological area or to the eyes and the orbits. Obstruction of the lacrimal pathway is a possible complication of the disease that affects approximately 7% of patients with WG. It usually occurs as a direct extension of sinonasal disease and typically is a late manifestation. Management of such a condition is generally viewed as difficult. We report the case of a patient with a quiescent WG limited to the otolaryngological area. This patient presented a bilateral obstruction of the nasolacrimal ducts caused by bilateral extensive adhesions in the nasal cavity. Because she had several episodes of left-side acute dacryocystitis which necessitated several courses of broad-spectrum antibiotics, she successfully underwent an endonasal endoscopic dacryocystorhinostomy using a diode laser and powered instrumentation. The authors describe the clinical case, the surgical technique, and review the literature.Keywords: Wegener’s granulomatosis, recurrent dacryocystitis, endonasal DCR, diode, laser, powered instrumentation

  19. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.

    Science.gov (United States)

    Shah, Shivani; Geetha, Duvuru

    2015-01-01

    Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction) and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility.

  20. Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient

    Directory of Open Access Journals (Sweden)

    Luna M

    2014-04-01

    Full Text Available Mariana Luna,1 Victoria Bocanegra,3 Patricia G Vallés1,21Nephrology Division, Pediatric Department, Dr Humberto Notti Pediatric Hospital, Mendoza, Argentina; 2Pathophysiology Area, Pathology Department, School of Medicine, National Cuyo University, Mendoza, Argentina; 3National Council of Scientific and Technical Research of Argentina (CONICET, Buenos Aires, ArgentinaAbstract: Granulomatosis with polyangiitis (GPA is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, hematological, and biochemical parameters improved substantially, achieving remission. Granulomatosis with polyangiitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and early aggressive immunosuppressive therapy are essential for the management of these patients.Keywords: acute renal failure, vasculitis, crescentic pauci-immune glomerulonephritis-Type-C, antineutrophil cytoplasmic antibodies (c-ANCA-macroscopic hematuria

  1. Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Robert Ali

    2015-01-01

    Full Text Available Granulomatosis with polyangiitis (GPA, previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

  2. 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 i

  3. 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...

  4. Cohort profile: the Welsh Geriatric Registrar-Led Research Network (WeGeN): rationale, design and description

    Science.gov (United States)

    Long, Sara; Butler, John

    2017-01-01

    Purpose Medical trainees are required to undertake audit and quality improvement projects. They must also have an understanding of the principles of research and are encouraged to participate in research projects. However, the constraints of time, a lack of formal training and rotation between different training posts create barriers to audit cycle completion and pursuing research. This leads to trainees being reluctant to undertake research, facilitates poor quality research and risks incomplete audit. Participants The Welsh Geriatricians Network (WeGeN) has been created with the aims of facilitating collaborative, trainee-led research within Geriatric Medicine in Wales, promoting research engagement and improving the research evidence base for older patients. By coordinating collaborative research projects across different sites within Wales, trainees continue existing projects at new sites, allowing completion of projects and establishing the long-term infrastructure and experienced personnel needed for high-quality research data to be gathered. Findings WeGeN has facilitated 4 national audits, all of which are intended for peer review publication. The first project considers the service provision for the older person in the emergency department, the second Parkinson's disease, the third reviews delirium management and the fourth project considers epidemiology of surgical disease in older people. Future plans The objective of this project is to further establish and develop WeGeN as a group which facilitates high-quality research and provides the opportunity for geriatric trainees to engage in research activity. It is anticipated that the establishment of this research platform will provide a blueprint for the development of other such networks in the UK and beyond. PMID:28196947

  5. 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

  6. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

    Directory of Open Access Journals (Sweden)

    Antoccia Antonio

    2010-04-01

    Full Text Available Abstract Ataxia-telangiectasia (A-T is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively with extensive cutaneous granulomatosis and severe combined immunodeficiency, without neurological abnormalities, at the time of diagnosis. This case clearly emphasizes the variable presentation of A-T syndrome and highlights the difficulties in the early diagnosis of A-T. A-T should be considered in children with evidence of combined humoral and cellular immunodeficiency associated with unexplained skin granulomatous lesions, even in the absence of the classic features of this syndrome.

  7. Granulomatosis de células de Langerhans: Presentación de un caso

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    . Caridad Nazco Ríos

    1998-12-01

    Full Text Available Se reporta un inusual caso de granulomatosis de células de Langerhans localizado en maxilares en un paciente de 30 meses de edad, quien presentaba además de las lesiones líticas óseas, lesiones de aspecto tumoral en encía palatina y lingual de ambas arcadas dentarias. El examen óseo no arrojaba compromiso de otros huesos. El diagnóstico histológico fue corroborado por el Centro Nacional de Referencia en Anatomía Patológica.A unusual case of Langerhans´ cells is reported, located in maxillae of a 30 months old patients, who, apart from bone lithic injuries, showed injuries of tumoral type in lingual and palatine gingiva of both dental archs. According to bone examination, there weren´t involvement of other bones. Histologic diagnosis was corroborated by National Reference Center in Pathologic Anatomy.

  8. Pronounced Risk of Fractures among Elderly Men Affected by Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Baslund, Bo; Obel, Niels

    2015-01-01

    OBJECTIVE: It is unknown whether patients affected by granulomatosis with polyangiitis (GPA) are at increased risk of fractures, and whether the fracture risk in GPA varies with age and sex. The aim of the present study was to compare the fracture risk among patients with GPA with that among age......- and sex-matched population controls. METHODS: We established a monocentric cohort of patients treated for GPA at a Danish tertiary care center from 1995 to 2010 (n = 159) and a register-derived GPA cohort identified from the Danish National Hospital Register (n = 402). Each patient was matched with 7...... population controls. The occurrence of fractures among patients was compared with that among controls by calculation of incidence rate ratios (IRR). RESULTS: In the monocentric cohort, an increased fracture risk was observed among men aged ≥ 55 years at the time of first hospitalization for GPA (IRR 3.5, 95...

  9. Risk of Diabetes Mellitus among Patients Diagnosed with Giant Cell Arteritis or Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Ahlström, Magnus G; Lindhardsen, Jesper

    2017-01-01

    patients diagnosed with giant cell arteritis (GCA) or granulomatosis with polyangiitis (GPA), i.e., patients with relatively common forms of systemic vasculitis. METHODS: We used Danish healthcare registries to identify 1682 patients diagnosed with GCA and 342 patients diagnosed with GPA from 1997 to 2015.......7-10.6) in the GPA cohort. During the first year after diagnosis of vasculitis, the IRR for DM was 7.0 (95% CI 5.2-9.3) among patients with GCA and 10.4 (95% CI 4.4-24) among patients with GPA. IRR for DM were not significantly increased in either cohort during later followup periods. Within the first year......, treatment with high cumulative prednisolone/PRED doses was associated with new-onset DM among the patients with vasculitis. CONCLUSION: Patients diagnosed with GCA or GPA have a markedly increased risk of new-onset DM during early treatment phases....

  10. Suppurative dacroadenitis causing ocular sicca syndrome in classic Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Khanna Dhanita

    2011-01-01

    Full Text Available Wegener′s granulomatosis (WG is a multisystem vasculitic disorder which can commonly afflict various components of the eye. Here we describe some unusual ocular manifestations of the disease in one patient. A young male with history of upper respiratory tract symptoms including epistaxis, nasal stuffiness and maxillary sinus pain presented with bilateral lacrimal gland abscess and ptosis. Lacrimal gland biopsy revealed granulomatous vasculitis. Lung cavities, positive cytoplasmic-antineutrophil cytoplasmic antibodies and high titers of serine proteinase-3 antibodies confirmed the diagnosis of WG. The patient developed dry eyes after a month of first presentation. There was no dryness of mouth, suggesting the absence of salivary gland involvement, and antinuclear antibodies as well as antibodies against Ro and La antigens classical of primary Sjogren′s syndrome were absent. Granulomatous vasculitis of lacrimal gland leading to abscess formation and dryness of eyes has not been described in WG and reflects the aggressive nature of inflammatory process in this disease.

  11. Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.

    Science.gov (United States)

    Iannella, Giannicola; Greco, Antonio; Granata, Guido; Manno, Alessandra; Pasquariello, Benedetta; Angeletti, Diletta; Didona, Dario; Magliulo, Giuseppe

    2016-07-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations.

  12. [Teutschlaender lipo-calcino-granulomatosis or tumoral calcinosis of Inclan (author's transl)].

    Science.gov (United States)

    Barrière, H; Welin, J; Lenne, Y; Visset, J; Vigier, P

    1977-02-01

    The present report describes a typical case of tumoral calcinosis. Differential diagnosis was initially directed towards sarcoma suggested by fibrous connective tissue surrounding cystlike cavities. The course of this disease led to chronic multiple fistulae with secondary infection in spite of two attempts of surgical excision. This case adds further support to the results of earlier reports showing no specific biological abnormalities. The present sutyd indicates otherwise that tumoral calcinosis and lipo-calcino-granulomatosis of Teutschlaender are the same condition. The pathogenesis of this affection remains enigmatic; there is no evidence that the lipidic excess should be the beginning of the process, but this disease must be considered as a distinctive form of calcinosis. The surgical exeresis is the only possible treatment, even if it doesn't prevent recurrence.

  13. Arthritis in lymphomatoid granulomatosis: Report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Agarwal Vikas

    2004-02-01

    Full Text Available Lymphomatoid granulomatosis (LG is a rare systemic vasculitis caused by Epstein Barr virus induced transformation of the B-cells in a T-cell rich environment. The predominant clinical presentations are confined to the pulmonary system however; extra-pulmonary manifestations can sometimes be the main feature of the disease. Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone. This is the first report of LG mimicking rheumatoid arthritis from India.

  14. Fatal Cytomegalovirus Disease after Combination Therapy with Corticosteroids and Rituximab for Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Talal Hilal

    2015-01-01

    Full Text Available The association of cytomegalovirus (CMV with autoimmune disease is poorly understood with suggested causality and reported viral reactivation coinciding with active inflammation. We report a case of a patient who presented with diffuse alveolar hemorrhage and acute renal failure from rapidly progressive glomerulonephritis ultimately diagnosed with granulomatosis with polyangiitis (GPA. She was acutely managed with plasmapheresis to reduce antibody-mediated end-organ damage, hemodialysis for worsening hyperkalemia and acidosis, and high-dose intravenous methylprednisolone. She was transitioned to oral prednisone and started on weekly rituximab with resultant remission induction over a three-week period at which point she developed reactivation of CMV causing severe fatal lung disease and viremia. The case highlights the multiple factors associated with CMV reactivation in cases of severe systemic inflammatory states and the need for further research to help establish practice guidelines regarding antimicrobial prophylaxis in patients with autoimmune diseases on prolonged courses of corticosteroids and biologic agents.

  15. Place in therapy of rituximab in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis

    Directory of Open Access Journals (Sweden)

    Shah S

    2015-08-01

    Full Text Available Shivani Shah, Duvuru Geetha Department of Medicine, Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, MD, USA Abstract: Granulomatosis with polyangiitis and microscopic polyangiitis are small vessel vasculitides characterized by circulating antineutrophil circulating antibodies. Standard treatment for active severe disease has consisted of cyclophosphamide with glucocorticoids with or without plasmapheresis, which achieves approximately 75% sustained remission, but carries significant adverse effects such as malignancy, infertility, leukopenia, and infections. The role of B cells in the pathogenesis of anti-neutrophil circulating antibodies-associated vasculitis has been established, and as such, rituximab, a monoclonal anti-CD20 antibody, has been studied in treatment of active granulomatosis with polyangiitis and microscopic polyangiitis (induction and in maintaining remission. Rituximab has been shown to be effective in inducing remission in several retrospective studies in patients with refractory disease or cyclophosphamide intolerance. The RAVE and RITUXVAS trials demonstrated rituximab is a noninferior alternative to standard cyclophosphamide-based therapy; however, its role in elderly patients and patients with severe renal disease warrants further investigation. Rituximab has been compared with azathioprine for maintaining remission in the MAINRITSAN trial and may be more efficacious in maintaining remission in patients treated with cyclophosphamide induction. Rituximab is not without risks and carries a similar adverse event risk rate as cyclophosphamide in randomized control trials. However, its use can be considered over cyclophosphamide in patients who have relapsing or refractory disease or in young patients seeking to preserve fertility. Keywords: rituximab, ANCA-associated vasculitis, GPA, MPA, induction therapy, maintenance therapy

  16. Altered Liver Proteoglycan/Glycosaminoglycan Structure as a Manifestation of Extracellular Matrix Remodeling upon BCG-induced Granulomatosis in Mice.

    Science.gov (United States)

    Kim, L B; Shkurupy, V A; Putyatina, A N

    2017-01-01

    Experimental BCG-induced granulomatosis in mice was used to study changes in the dynamics of individual liver proteoglycan components reflecting phasic extracellular matrix remodeling, determined by the host-parasite interaction and associated with granuloma development. In the early BCG-granulomatosis period, the increase in individual proteoglycan components promotes granuloma formation, providing conditions for mycobacteria adhesion to host cells, migration of phagocytic cells from circulation, and cell-cell interaction leading to granuloma development and fibrosis. Later, reduced reserve capacity of the extracellular matrix, development of interstitial fibrosis and granuloma fibrosis can lead to trophic shortage for cells within the granulomas, migration of macrophages out of them, and development of spontaneous necrosis and apoptosis typical of tuberculosis.

  17. How Earth works 100 years after Wegener's continental drift theory and IGCP 648

    Science.gov (United States)

    Li, Z. X.; Evans, D. A.; Zhong, S.; Eglington, B. M.

    2015-12-01

    It took half a century for Wegener's continental drift theory to be accepted as a fundamental element of the plate tectonic theory. Another half a century on, we are still unsure of the driving mechanism for plate tectonics: is it dominated by thermal convection, gravitational forces, or by a combination of mechanisms? Nonetheless, breakthroughs in the past decades put us in a position to make a major stride in answering this question. These include: (1) widely accepted cyclic occurrences of supercontinent assembly and break-up (whereas random occurrence of supercontinents was an equal possibility in the 1990s); (2) the discovery of two equatorial and antipodal large low seismic velocity provinces (LLSVPs) that dominate the lower mantle and appear to have been the base for almost all mantle plumes since at the Mesozoic, and of subduction of oceanic slabs all the way to the core-mantle boundary, which together suggesting whole-mantle convection; (3) the recognition of true polar wander (TPW) as an important process in Earth history, likely reflecting Earth's major internal mass redistribution events; and (4) rapidly enhancing computer modelling power enabling us to simulate all aspect of Earth's dynamic inner working. Many new yet often controversial ideas have been proposed, such a possible coupling in time (with an offset) and space between supercontinent cycle and superplume (LLSVP) events which oppose to the idea of static and long-lived LLSVPs, and the orthoversion v.s. introversion or extroversion models for supercontinent transition. To fully utilise these advances as well as the rapidly expanding global geoscience databases to address the question of how Earth works, an UNESCO-IUGS sponsored IGCP project No. 648 was formed to coordinate a global cross-disciplinary effort. We aim to achieve a better understanding of the supercontinent cycle, and examine the relationship between supercontinent cycle and global plume events. We will establish a series of global

  18. Hémothorax spontané révélant une vascularite de Wegener chez une femme enceinte

    Science.gov (United States)

    Serhane, Hind; Yassine, Msougar; Amro, Lamyae

    2016-01-01

    L’hémothorax spontané demeure une pathologie rare. Ses étiologies sont multiples mais restent parfois inconnues. Chez certains patients, la thoracotomie peut être le seul recours pour déterminer son origine. Les vascularites n’ont pas été rapporté comme cause habituelle des hémothorax spontanés. La grossesse ne semble pas avoir d’effet causal, ni aggravant des hémothorax spontanés, ni des vascularites. Nous rapportons une observation assez particulière d’une jeune patiente, présentant au cours de sa grossesse un hémothorax spontané secondaire à une vasularite de type Wegener, diagnostiquée par la biopsie pleurale faite au cours d’une thoracotomie exploratrice et confirmée par le dosage des ANCA. PMID:28154726

  19. MicroRNA-941 Expression in Polymorphonuclear Granulocytes Is Not Related to Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Svendsen, Jesper Brink; Baslund, Bo; Cramer, Elisabeth Præstekjær

    2016-01-01

    granulomatosis with polyangiitis (GPA) have lower expression of miR-941 than healthy control donors as a biological cause for higher JMJD3 levels. We found no significant difference in the degree of maturation of PMNs from GPA patients (n = 8) and healthy controls (n = 11) as determined from cell surface...... expression of the neutrophil maturation marker CD16 and gene expression profile of FCGR3B. The expression of PRTN3 and KDM6B mRNAs and miR-941 was not significantly different in GPA patients and healthy controls. Transfection of pre-miR-941 into the neutrophil promyelocyte cell line PLB-985 cells did...... not result in reduction of the KDM6B mRNA level as shown previously in a hepatocellular carcinoma cell line. The amount of PR3 in PMNs from GPA patients and healthy controls was comparable. In conclusion, we found that PRTN3 mRNA, KDM6B mRNA, and miR-941 expression levels in PMNs do not differ between GPA...

  20. Impact of pre-existing co-morbidities on mortality in granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Ahlström, Magnus Glindvad; Lindhardsen, Jesper

    2016-01-01

    OBJECTIVE: To assess the impact of pre-existing co-morbidities on mortality among patients affected by granulomatosis with polyangiitis (GPA). METHODS: By means of the Danish National Hospital Register, we identified a cohort of patients hospitalized for GPA during 1994-2010 (n = 308). The burden...... throughout 2010. Cox regression analyses were used to calculate mortality rate ratios (MRRs). RESULTS: The median duration of follow-up in the GPA cohort was 5.8 years (interquartile range 2.3-10.0). Compared with their matched population controls, the MRR for patients presenting with a CCI score of 0 (n...... = 246) was 3.9 (95% CI 2.0, 7.5) during years 0-2 and 1.4 (95% CI 0.9, 2.0) from the second year of follow-up onwards. The corresponding MRRs were 13.3 (95% CI 5.8, 31) and 1.9 (95% CI 1.1, 3.6) for patients with a CCI score ⩾1 (n = 62). In a direct comparison, GPA patients with a CCI score ⩾1 were...

  1. Eosinophilic granulomatosis with polyangiitis complicated by cholecystitis: a case report and review of the literature.

    Science.gov (United States)

    Ye, Lu; Lu, Xiaoyong; Xue, Jing

    2016-01-01

    The objectives are to report an atypical case of eosinophilic granulomatosis with polyangiitis (EGPA) associated with cholecystitis and to review the main clinical features, therapy, and prognosis of it. We present a 49-year-old male with non-classic clinical manifestations of EGPA and EGPA-related cholecystitis. EGPA was diagnosed by histology of the gallbladder after cholecystectomy. In addition, 11 cases of EGPA-associated cholecystitis have been reported and were described in details in this article. Gallbladder involvement is very uncommon in EGPA. All cases reviewed showed multiple organs involved as well as obviously elevated eosinophilic granulocyte proportion with inflammatory index, although antineutrophil cytoplasmic antibody may be negative. All patients in this cohort that showed gallbladder involvement were eventually confirmed with EGPA by histology examination after cholecystectomy. The pathological change could be infiltration of inflammatory mononuclear cells of small- and medium-sized vessels. Of the cases, 91.7% responded well to steroid and immunosuppressant therapy. Gallbladder involvement is a very rare comorbid condition in EGPA. However, it is an important symptom or secondary condition to alert physicians the diagnosis of EGPA. Moreover, timely diagnosis and correct administration in the early stage of this disease could obviously improve the prognosis.

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

    Directory of Open Access Journals (Sweden)

    Gulazyk Malikovna Koilubaeva

    2014-01-01

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

  3. A case of exorbitism in association with Wegener′s granulomatosis with renal involvement

    Directory of Open Access Journals (Sweden)

    S Beji

    2012-01-01

    Full Text Available Wegener′s granulomatosis (WG is a necrotizing granulomatous vasculitis invol-ving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glo-merulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bila-teral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

  4. Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.

    Science.gov (United States)

    Furuta, Shunsuke; Chaudhry, Afzal N; Arimura, Yoshihiro; Dobashi, Hiroaki; Fujimoto, Shouichi; Homma, Sakae; Rasmussen, Niels; Jayne, David R

    2017-02-01

    There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan. We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse. Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p UK (61.0% vs 85.2%, p UK (34.1% vs 8.6%, p UK (68.1 μmol/l vs 101.0 μmol/l, p UK (69.5% vs 40.6%, p UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.

  5. A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis: case report and literature review

    Directory of Open Access Journals (Sweden)

    FU Yong-juan

    2012-10-01

    Full Text Available Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG. Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI showed space-occupying lesions in bilateral frontal lobes, with T1WI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein-Barr virus (EBV was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.

  6. [Sarcoidosis--granulomatosis: the modern view of the etiology and pathogenesis with clinical cases review].

    Science.gov (United States)

    Kazmirchuk, V E; Tsarik, V V; Mal'tsev, D V; Ishchenko, M I

    2014-01-01

    Diagnostic criteria of sarcoidosis were offered in the 60-ies of XX century, however today the problem of sarcoidosis is difficult for understanding the different specialists and early detection. The development of laboratory diagnostic of viral infections and introduction of polymerase chain reaction (PCR) has greatly improved the level of diagnosis of herpes infections, reveal the previously unknown etiology of many diseases: sarcoidosis (granulomatosis), migraine, multiple sclerosis, cystic prenatal brain damage, convulsions, Hodgkin's disease and others. Sarcoidosis is the set of clinical symptoms (fatigue, shortness of breath, coughing, heaviness in the chest), laboratory, radiological and histopathological data that allow the doctor to diagnose, predict prognosis and treatment policy. Most often, sarcoidosis affects the lungs and thoracic lymph nodes. In the last period for 2011-2013 in 2930 immunologically tested patients the sarcoidosis was confirmed in 146. Primarily these patients were exposed to different diagnosis--COPD, pneumonia, tuberculosis, lung cancer. Among patients with sarcoidosis on the first place in frequency of detection were EBV and HHV-6. We were the first in 2000, described the Epstein-Barr virus as the causative factor of sarcoidosis, and has been hypothesized the immunopathology of sarcoidosis and principles of individual immunotherapy with a resolution of the granulomatous process in 92 % of cases. Subsequently, this association has been and illustrates the relationship to other viruses (HHV-6, HHV-8) demonstrated by other authors.

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

    Directory of Open Access Journals (Sweden)

    Ryan Kunjal

    2016-01-01

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

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

    Science.gov (United States)

    Kunjal, Ryan; Makary, Raafat; Poenariu, Andreea

    2016-01-01

    Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis rarely affects females of reproductive age. A 28-year-old African American woman presented at 8 weeks of gestation with intractable vomiting attributed to hyperemesis gravidarum. She was found to have acute kidney injury that was unresponsive to vigorous fluid resuscitation and urine sediment examination was suggestive of an underlying glomerulonephritis. Serum c-ANCA and PR3 were elevated and there was no peripheral eosinophilia. During her course she also developed one episode of small volume hemoptysis with right upper lobe infiltrates on CT Chest. There were no cutaneous manifestations of vasculitis or upper respiratory symptoms. Renal biopsy revealed a pauci-immune crescentic glomerulonephritis (PICGN). The diagnosis was consistent with granulomatosis with polyangiitis (GPA). Management initially comprised teratogen sparing agents; steroids, intravenous immunoglobulin; and plasma exchange. The response was suboptimal and she became dependent on daily renal replacement therapy. Ultimately the pregnancy was terminated allowing for traditional treatment approaches with dramatic effect. This is the first case of GPA presenting as PICGN in pregnancy and highlights the challenges of its management. PMID:27293925

  9. Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss.

    Directory of Open Access Journals (Sweden)

    Christian Dejaco

    Full Text Available Previous studies suggest a role for eotaxin-3, TARC/CCL17 and IgG4 in newly-diagnosed patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss with highly active disease. The role of these biomarkers in relapsing disease is unclear.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio were determined in serum samples from a longitudinal cohort of patients with EGPA (105 visits of 25 patients. Epidemiological, clinical and laboratory data were available for all visits.At the first visit, 80% of patients were using glucocorticoids and 68% additional immunosuppressive drugs. Disease flares were seen at 18 visits. The median BVAS and BVAS/WG scores at time of relapse were 4 and 2, respectively. None of the biomarkers tested were useful to discriminate between active disease and remission. Patients treated with prednisone had lower eotaxin-3 and eosinophil levels compared to patients not taking glucocorticoids irrespective of disease activity. Use of immunosuppressive agents was not associated with biomarker levels.Serum levels of TARC/CCL17, eotaxin-3, IgG4, and IgG4/IgG ratio do not clearly differentiate active and inactive disease in established EGPA. Defining biomarkers in EGPA remains a challenge especially during times of glucocorticoid use.

  10. DOCK 8 Deficiency, EBV+ Lymphomatoid Granulomatosis, and Intrafamilial Variation in Presentation.

    Science.gov (United States)

    Dimitriades, Victoria R; Devlin, Vincent; Pittaluga, Stefania; Su, Helen C; Holland, Steven M; Wilson, Wyndham; Dunleavy, Kieron; Shah, Nirali N; Freeman, Alexandra F

    2017-01-01

    Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections. She had normal immunoglobulins with elevated IgE, poor polysaccharide response with low switched memory B cells, low CD4 count, and normal mitogen and antigen responses. Despite clinical improvement following immunoglobulin replacement, a prolonged cough prompted a CT scan, which showed nodules. Biopsy identified a Grade 2 EBV + LYG. Due to an inadequate response with chemotherapy, further workup for primary immunodeficiency was performed. With her symptoms of eczema and IgE elevation, along with her brother's history of recurrent sinopulmonary infections and warts, targeted sequencing of DOCK8 was performed revealing compound heterozygous mutations for the two siblings. Both patients were successfully transplanted with resolution of the LYG and warts, respectively. This is the first reported case of LYG in DOCK8 deficiency. The EBV-driven lymphoproliferative disease along with the infection history in the brother led to the diagnosis of DOCK8 deficiency and curative hematopoietic stem cell transplants.

  11. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Mellemkjaer, Lene; Voss, Anne

    2015-01-01

    -melanoma skin cancers (NMSCs) and 11 were bladder carcinomas. A high occurrence of NMSC was observed from the second year of follow-up onwards, with a SIR of 7.0 (95% CI 2.3, 16) for cases diagnosed ≥20 years after GPA. The incidence of bladder cancer increased after 5-9, 10-14 and 15-19 years of follow......OBJECTIVE: The long-term cancer risk for patients treated for granulomatosis with polyangiitis (GPA) is not well characterized. We assessed the risk of early and late-occurring cancers among 293 patients diagnosed with GPA from 1973 to 1999 and followed throughout 2010. METHODS: Cancer incidence...... in the cohort was determined by linkage with the Danish Cancer Registry and compared with that in the general population by calculation of standardized incidence ratios (SIRs). RESULTS: The median duration of follow-up was 9.7 years (range 0-36). Seventy-three cancers occurred, of which 30 were non...

  12. Generation of high-resolution wind fields from the dense meteorological station network WegenerNet in South-Eastern Austria

    Science.gov (United States)

    Schlager, Christoph; Kirchengast, Gottfried; Fuchsberger, Jürgen

    2016-04-01

    To investigate weather and climate on a local scale as well as for evaluating regional climate models (RCMs) the Wegener Center at the University of Graz established the long-term field experiment WegenerNet Feldbach region, a dense grid of 153 meteorological stations. The observations of these stations are managed by an automatic WegenerNet Processing system. This system includes a quality check of collected observations and a Data Product Generator (DPG), among other subsystems. Products already implemented in the DPG are gridded weather and climate products, generated from the main parameters temperature, precipitation and relative humidity (Kirchengast et. al., Bull. Amer. Meteor. Soc., 95, 227-242, 2014). Missing elements are gridded wind fields from wind observations. Wind is considered as one of the most difficult meteorological variables to model and depends on many different parameters such as topography and surface roughness. Therefore a simple interpolation can only be performed in case of uniform characteristics of landscape. The presentation introduces our method of generation of wind fields from near real-time observations of the WegenerNet. Purpose of this work is to provide a database with 3D wind fields in a high spatial and time resolution as addition to the existing products, for evaluating convection permitting climate models as well as investigating weather and climate on a local scale. Core of the application is the diagnostic California Meteorological Model (CALMET). This model computes 3D wind fields based on meteorological observational data, a digital elevation model and land use categories. The application generates the required input files from meteorological stations of the WegenerNet Feldbach region and triggers the start of the CALMET model with these input files. In a next step the modeled wind fields are stored automatically every 30 minutes with a spatial resolution of 100 x 100 m in the WegenerNet database. To verify the

  13. 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.

  14. Successful Use of Plasma Exchange in the Treatment of Corticosteroid-Refractory Eosinophilic Granulomatosis with Polyangiitis Associated with Gastrointestinal Manifestations

    Directory of Open Access Journals (Sweden)

    Kohei Tsujimoto

    2016-01-01

    Full Text Available We describe the case of a 33-year-old woman having corticosteroid-refractory eosinophilic granulomatosis with polyangiitis (EGPA who presented with abdominal pain and responded dramatically to plasma exchange. Eosinophilia, asthma history, neuropathy, pulmonary infiltrates, and paranasal sinus abnormalities confirmed the diagnosis of EGPA. Treatment was initiated with 1 g/day of methylprednisolone pulse therapy for 3 days followed by 60 mg/day of intravenous prednisolone without relieving abdominal pain. Then, plasma exchange was performed thrice. Abdominal pain disappeared after the first plasma exchange. Indication of plasma exchange for EGPA remains controversial; however, it may represent a valid option in cases with gastrointestinal involvement.

  15. Genetics and pathophysiology of granulomatosis with polyangiitis (GPA) and its main autoantigen proteinase 3.

    Science.gov (United States)

    Relle, Manfred; Föhr, Bernd; Fasola, Federica; Schwarting, Andreas

    2016-12-01

    Granulomatosis with polyangiitis (GPA) is a severe autoimmune disease and one of the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although its etiology and pathophysiology are still widely unknown, it is accepted that infections, environmental factors, epigenetic modifications, and a genetic predisposition provide the basis for this systemic disorder. GPA typically evolves into two phases: an initial phase characterized by ear, nose and throat (ENT) manifestations, such as chronic sinusitis and otitis, ulceration of the oral cavity and pharynx, as well as pulmonary nodules and a severe generalized phase, defined by the occurrence of rapidly progressive glomerulonephritis, pulmonary hemorrhage, and arthritis. ANCAs, directed against the neutrophilic enzymes proteinase 3 and myeloperoxidase, are present in up to 90% of the affected patients in the systemic phase. As the humoral immunity is predominantly directed against neutrophilic antigens, it is apparent that neutrophils play a critical role in GPA both as target and effector cells. Although GPA pathogenesis is not well known, some susceptibility genes and loci have been identified by candidate gene approaches, genome-wide association studies, and meta-analyses, as well as familial association studies. Such genes are CTLA4, PTPN22, COL11A2, SERPINA1, and the MHC class II gene cluster. This review highlights the clinical, pathophysiological, and genetic background of GPA and aims to give an overview of recent efforts to identify GPA susceptibility genes. We point out the genetic basis of the main autoantigen PR3 and why it is so difficult to establish a murine GPA model. Copyright © 2016 Elsevier Ltd. All rights reserved.

  16. IgA antibodies to myeloperoxidase in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

    Science.gov (United States)

    Oommen, Esha; Hummel, Amber; Allmannsberger, Lisa; Cuthbertson, David; Carette, Simon; Pagnoux, Christian; Hoffman, Gary S; Jenne, Dieter E; Khalidi, Nader A; Koening, Curry L; Langford, Carol A; McAlear, Carol A; Moreland, Larry; Seo, Philip; Sreih, Antoine; Ytterberg, Steven R; Merkel, Peter A; Specks, Ulrich; Monach, Paul A

    2017-01-01

    To determine the prevalence of anti-myeloperoxidase (MPO) antibodies of IgA (IgA anti-MPO) isotype in patients with eosinophilic granulomatosis with polyangiitis (EGPA), and the association of the IgA antibodies with IgG anti-MPO and with disease activity. Serum samples from patients with EGPA followed in a multicenter longitudinal cohort were tested by ELISA for the presence of IgA anti-MPO and IgG anti-MPO antibodies. Sera from 87 healthy controls were used to define a positive test. Sera from 168 patients with EGPA (298 samples) were tested. Frequencies of positive testing for IgA anti-MPO were compared between patients with active EGPA, patients in remission, and controls. IgA anti-MPO was detected in 10 of 168 (6%) patients with EGPA (11 of 298 serum samples) compared to 1 of 87 (1%) healthy controls (p=0.10). All 11 samples testing positive for IgA anti-MPO also tested positive for IgG anti-MPO. Ninety samples tested positive for IgG anti-MPO but negative for IgA. Samples taken during active EGPA were positive for IgA anti-MPO in 6/72 cases (8%), compared to 5/226 (2%) during remission (p=0.03). Among samples taken during moderate or high disease activity, 5/41 were positive (12%, p=0.01 compared to remission). Although IgA anti-MPO antibodies are detectable in some patients with EGPA and may be detectable more frequently during active disease, their presence seems unlikely to provide information beyond what is obtained from conventional IgG anti-MPO.

  17. Azathioprine is effective for oral involvement in Crohn's disease but not for orofacial granulomatosis alone.

    Science.gov (United States)

    Mentzer, A; Goel, R; Elliott, T; Campbell, H; Hullah, E; Patel, P; Challacombe, S; Escudier, M; Sanderson, J D

    2016-04-01

    There have been no previous reports assessing the effectiveness of azathioprine (AZA) in the treatment of orofacial granulomatosis (OFG). This report is a review of patients receiving AZA for active OFG with or without concomitant gut Crohn's disease (CD) in a specialist tertiary referral centre. Clinical response was defined by Global Physician Assessment at 4-, 12- and 24-month follow-up and a standardised oral disease activity score (ODAS). Sixty of 215 patients seen with OFG in our clinic over a 12-year period were treated with AZA. Of these, 22 had concomitant CD. The proportion of patients responding to AZA with a diagnosis of CD/OFG vs. OFG only at 4, 12 and 24 months were 54% vs. 21% (P = 0.03), 59% vs. 21% (P = 0.003) and 41% vs. 24% (P = 0.16), respectively. A statistically significant difference was seen between starting and follow-up ODAS scores at 4 months in the CD/OFG group which was not observed in the OFG only group. Factors predicting a need for AZA included a diagnosis of intestinal CD, sulcal swelling, sulcal ulcers and upper lip involvement. The factor predicting response to treatment was a diagnosis of CD at 12 months of follow-up. No difference in the number of adverse effects was observed between the two groups of patients. AZA is significantly more effective in the treatment of oral disease with a concurrent diagnosis of CD rather than in the treatment of OFG alone. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. Vasculitis Pregnancy Registry

    Science.gov (United States)

    2016-09-08

    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

  19. Aortic {sup 18}F-FDG uptake in patients suffering from granulomatosis with polyangiitis

    Energy Technology Data Exchange (ETDEWEB)

    Kemna, Michael J. [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Bucerius, Jan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); University Hospital RWTH Aachen, Department of Nuclear Medicine, Aachen (Germany); Drent, Marjolein [Maastricht University, Department of Pharmacology and Toxicology, Maastricht (Netherlands); Voeoe, Stefan [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands); Veenman, Martine [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Paassen, Pieter van [Maastricht University Medical Center, Department of Nephrology and Clinical Immunology, Maastricht (Netherlands); Tervaert, Jan Willem Cohen [Maastricht University, Cardiovascular Research Institute Maastricht (CARIM), Maastricht (Netherlands); Sint Franciscus Gasthuis, Noordoever Academy, Rotterdam (Netherlands); Kroonenburgh, Marinus J.P.G. van [Maastricht University Medical Center, Department of Nuclear Medicine, Maastricht (Netherlands)

    2015-08-15

    The objective of the study was to systematically assess aortic inflammation in patients with granulomatosis with polyangiitis (GPA) using {sup 18}F-2-deoxy-2-[{sup 18}F]fluoro-D-glucose (FDG) positron emission tomography (PET)/CT. Aortic inflammation was studied in PET/CT scans obtained from 21 patients with GPA; 14 patients with sarcoidosis were included as disease controls, 7 patients with stage I or II head and neck carcinoma ascertained during routine clinical practice were used as healthy controls (HC) and 5 patients with large vessel vasculitis (LVV) were used as positive controls. Aortic {sup 18}F-FDG uptake was expressed as the blood-normalized maximum standardized uptake value (SUV{sub max}), known as the target to background ratio (mean TBR{sub max}). The mean TBR{sub max} (interquartile range) of the aorta in patients with GPA, sarcoidosis, HC and LVV were 1.75 (1.32-2.05), 1.62 (1.54-1.74), 1.29 (1.22-1.52) and 2.03 (1.67-2.45), respectively. The mean TBR{sub max} was significantly higher in patients suffering from GPA or LVV compared to HC (p < 0.05 and p < 0.005, respectively) and tended to be higher in patients suffering from sarcoidosis, but this did not reach statistical significance (p = 0.098). The mean TBR{sub max} of the most diseased segment was significantly higher compared to HC [1.57 (1.39-1.81)] in LVV patients [2.55 (2.22-2.82), p < 0.005], GPA patients [2.17 (1.89-2.83), p < 0.005] and patients suffering from sarcoidosis [2.04 (1.88-2.20), p < 0.05]. In GPA patients, the mean TBR{sub max} of the aorta was significantly higher in patients with previous renal involvement [2.01 (1.69-2.53)] compared to patients without renal involvement in the past [1.60 (1.51-1.80), p < 0.05]. Interrater reproducibility with a second reader was high (all intraclass correlation coefficients >0.9). Patients suffering from GPA show marked aortic FDG uptake. (orig.)

  20. MicroRNA-941 Expression in Polymorphonuclear Granulocytes Is Not Related to Granulomatosis with Polyangiitis

    Science.gov (United States)

    Svendsen, Jesper Brink; Baslund, Bo; Cramer, Elisabeth Præstekjær; Rapin, Nicolas; Borregaard, Niels

    2016-01-01

    Jumonji Domain-Containing Protein 3 (JMJD3)/lysine demethylase 6B (KDM6B) is an epigenetic modulator that removes repressive histone marks on genes. Expression of KDM6B mRNA is elevated in leukocytes from patients with ANCA-associated vasculitis (AAV) and has been suggested to be the reason for higher proteinase 3 (PR3) mRNA expression in these cells due to derepression of PRTN3 gene transcription. MicroRNA-941 (miR-941) has been shown to target KDM6B mRNA and inhibit JMJD3 production. We therefore investigated whether polymorphonuclear granulocytes (PMNs) from patients suffering from granulomatosis with polyangiitis (GPA) have lower expression of miR-941 than healthy control donors as a biological cause for higher JMJD3 levels. We found no significant difference in the degree of maturation of PMNs from GPA patients (n = 8) and healthy controls (n = 11) as determined from cell surface expression of the neutrophil maturation marker CD16 and gene expression profile of FCGR3B. The expression of PRTN3 and KDM6B mRNAs and miR-941 was not significantly different in GPA patients and healthy controls. Transfection of pre-miR-941 into the neutrophil promyelocyte cell line PLB-985 cells did not result in reduction of the KDM6B mRNA level as shown previously in a hepatocellular carcinoma cell line. The amount of PR3 in PMNs from GPA patients and healthy controls was comparable. In conclusion, we found that PRTN3 mRNA, KDM6B mRNA, and miR-941 expression levels in PMNs do not differ between GPA patients and healthy controls, and that miR-941 does not uniformly regulate KDM6B mRNA levels by inducing degradation of the transcript. Thus, decreased miR-941 expression in PMNs cannot be part of the pathogenesis of GPA. PMID:27755585

  1. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.

    Science.gov (United States)

    Lazzerini, Marzia; Bramuzzo, Matteo; Ventura, Alessandro

    2014-06-21

    To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn's disease. We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (< 18 years), with no language limitations. All relevant articles were accessed in full text. The manual search included references of retrieved articles. We extracted data on patients' characteristics, disease characteristics, association with other diseases, and treatment. We analyzed the data and reported the results in tables and text. We retrieved 173 reports of OFG in children. Mean age at onset was 11.1 ± 3.8 years (range: 2.0-18 years). Prevalence in males was significant higher than in females (P < 0.001), with a male:female ratio of 2:1. Gastrointestinal signs or symptoms were present in 26.0% of children at the time of OFG diagnosis. Overall, 70/173 (40.4%) children received a concomitant diagnosis of Crohn's disease. In about half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn's disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn's disease. Both perianal disease and a family history of Crohn's disease were significantly associated with a higher risk of Crohn's disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74, 95%CI: 2.24-3.36, P < 0.0001 for both). Treatment of OFG included steroids (70.8% of children) and other immunosuppressive drugs (42.7%), such as azathioprine, thalidomide and infliximab. High prevalence of Crohn's disease in children with OFG suggests that OFG may be a subtype of Crohn's disease.

  2. WegenerNet climate station network region Feldbach/Austria: From local measurements to weather and climate data products at 1 km-scale resolution

    Science.gov (United States)

    Kabas, T.; Leuprecht, A.; Bichler, C.; Kirchengast, G.

    2010-12-01

    South-eastern Austria is characteristic for experiencing a rich variety of weather and climate patterns. For this reason, the county of Feldbach was selected by the Wegener Center as a focus area for a pioneering observation experiment at very high resolution: The WegenerNet climate station network (in brief WegenerNet) comprises 151 meteorological stations within an area of about 20 km × 15 km (~ 1.4 km × 1.4 km station grid). All stations measure the main parameters temperature, humidity and precipitation with 5 minute sampling. Selected further stations include measurements of wind speed and direction completed by soil parameters as well as air pressure and net radiation. The collected data is integrated in an automatic processing system including data transfer, quality control, product generation, and visualization. Each station is equipped with an internet-attached data logger and the measurements are transferred as binary files via GPRS to the WegenerNet server in 1 hour intervals. The incoming raw data files of measured parameters as well as several operating values of the data logger are stored in a relational database (PostgreSQL). Next, the raw data pass the Quality Control System (QCS) in which the data are checked for its technical and physical plausibility (e.g., sensor specifications, temporal and spatial variability). In consideration of the data quality (quality flag), the Data Product Generator (DPG) results in weather and climate data products on various temporal scales (from 5 min to annual) for single stations and regular grids. Gridded data are derived by vertical scaling and squared inverse distance interpolation (1 km × 1 km and 0.01° × 0.01° grids). Both subsystems (QCS and DPG) are realized by the programming language Python. For application purposes the resulting data products are available via the bi-lingual (dt, en) WegenerNet data portal (www.wegenernet.org). At this time, the main interface is still online in a system in which

  3. The use of abatacept in debilitating cavitating lung disease associated with rheumatoid arthritis, bronchocentric granulomatosis and aspergillosis.

    LENUS (Irish Health Repository)

    Neff, K

    2010-06-01

    A case of debilitating cavitating lung disease associated with rheumatoid arthritis and bronchocentric granulomatosis, which failed to respond to conventional medical or surgical treatment, is described. The patient was treated over 10 years with steroids, antimicrobial agents, disease-modifying antirheumatoid drugs and surgery. Lung function continued to decline and the patient presented for admission with recurrent pneumonia. Abatacept was initiated to modify the underlying immunopathology. Following 12 months of treatment with abatacept the patient has demonstrable improvement in lung function and lung anatomy, and has not presented to hospital with pneumonia. She has tolerated the treatment without complication. The use of abatacept has stabilised the lung disease in this case in the medium term and prevented readmission to hospital. These results suggest a larger role for abatacept in those with such disease in the future and may warrant further investigation.

  4. [Hereditary systemic autoinflammatory diseases. Part II: cryopyrin-associated periodic syndromes, pediatric systemic granulomatosis and PAPA syndrome].

    Science.gov (United States)

    Aróstegui, Juan I; Yagüe, Jordi

    2008-03-29

    Hereditary systemic autoinflammatory diseases result from a genetically-based dysregulated inflammatory process, and are clinically characterized by recurrent or persistent systemic inflammatory episodes, which typically occur in the absence of infectious, neoplastic or autoimmune etiology. Elucidation of their molecular basis has enabled the use of genetic analyses to achieve an accurate and definitive diagnosis, and to establish a tailored treatment. The present review is the second and last part of an updated and comprehensive overview of hereditary systemic autoinflammatory diseases, and will introduce persistent, non-periodic autoinflammatory diseases, such as: a) the group of cryopyrin-associated periodic syndromes (CAPS), which includes familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells syndrome, and CINCA-NOMID syndrome; b) the group of pediatric systemic granulomatosis, which includes both Blau syndrome and early-onset sarcoidosis, and c) the pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) syndrome.

  5. Lymphomatoid Granulomatosis of Central Nervous System and Lung Driven by Epstein Barr Virus Proliferation: Successful Treatment with Rituximab

    Directory of Open Access Journals (Sweden)

    Ruben Fernandez

    2014-02-01

    Full Text Available Lymphomatoid granulomatosis (LYG is a very rare Epstein-Barr virus (EBV associated B-cell lymphoproliferative disorder. We report the case of a 41-year-old man who presented with fever and respiratory symptoms. Computed tomography showed multiple nodules in both lung fields. Polymerase chain reaction (PCR analysis of the bronchoalveolar lavage was positive for EBV and biopsy of lung node yielded a diagnosis of LYG, grade III. Shortly after initiation of treatment with chemotherapy, neurologic deterioration appeared. Radiologic findings revealed hydrocephalus and PCR analysis of the cerebrospinal fluid (CSF was positive for EBV. Treatment with intravenous rituximab led to rapid reduction of EBV load in CSF, along with radiological and clinical improvement. After completion of treatment with immunochemotherapy, an autologous stem cell transplantation was performed. Patient stays in remission one year after diagnosis.

  6. Depositional evolution of a reef-dominated Upper Permian carbonate platform, Wegener Halvoe Formation, Karstryggen area, East Greenland

    Energy Technology Data Exchange (ETDEWEB)

    Stemmrik, L.

    1996-12-31

    The Wegener Halvoe Formation carbonate platform in the Karstryggen area consists of three third order depositional sequences that formed in response to three Kazanian sea level cycles. Pinning point curves for the sub-aerial exposure surfaces separating the depositional sequences quantify the amplitude of the relative sea level fluctuations in the range of 70-140 m. The Karstryggen platform developed on top of a karstified older Permian carbonate platform and pre-depositional relief was locally exceeding 70 m. The pre-depositional relief influenced deposition in all three sequences. Algal-cement buildups formed over karst pinnacles and merged during time to form a N-S trending barrier dissected by deep fluvially modified karst channels. Peritidal carbonates formed behind the barrier whereas off platform sedimentation was dominated by thin transgressive siliciclastics overlain by shallow marine algal-foraminifer grain-stones in the first two sequences. During the last sea level cycle pro-grading oolitic grain-stones formed basin-wards of the algal-cement buildups. Transgressive systems tracts are thin and often dominated by condensed siliciclastic units in off platform areas and palaeolows. Over palaeotopographic highs it consists of aggrading algal cement-stones. High-stand deposits are limited to palaeotopographic elevated areas and consist of algal cementstones along the basin margin and shallow sub-tidal to inter-tidal carbonates and evaporites in the platform area. Outside these areas carbonate deposition took place during falling sea level, and during deposition of the first two sequences thin laterally extensive units of shallow marine grain-stones were deposited directly on top of deeper marine siliciclastics. During the final sea level fall, thick pro-grading units of oolitic grain-stones were deposited. (au) Appendix no. 3. 28 refs.

  7. Woorden wikken en wegen

    NARCIS (Netherlands)

    Kraaij, W.

    2009-01-01

    Zoals eenieder zich nog wel de roes kan herinneren na de eindexamenuitslag van de middelbare school, zo heeft voor mij ook de eerste kennismaking met het world wide web een bijzondere indruk gemaakt. Het zal ergens in 1993 geweest zijn toen ik aan collega’s van het toenmalige Instituut voor Taal en

  8. Prolonged B cell depletion with rituximab is effective in treating refractory pulmonary granulomatous inflammation in granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Henderson, Scott R; Copley, Susan J; Pusey, Charles D; Ind, Philip W; Salama, Alan D

    2014-12-01

    Pulmonary nodule formation is a frequent feature of granulomatosis with polyangiitis (GPA). Traditional induction therapy includes methotrexate or cyclophosphamide, however, pulmonary nodules generally respond slower than vasculitic components of disease. Efficacy of rituximab (RTX) solely for the treatment of pulmonary nodules has not been assessed. In this observational cohort study, we report patient outcomes with RTX in GPA patients with pulmonary nodules who failed to achieve remission following conventional immunosuppression. Patients (n = 5) with persistent pulmonary nodules were identified from our clinic database and retrospectively evaluated. Systemic manifestations, inflammatory markers, disease activity, concurrent immunosuppression, and absolute B cell numbers were recorded pre-RTX and at 6 monthly intervals following treatment. Chest radiographs at each time point were scored by an experienced radiologist, blinded to clinical details. Five patients with GPA and PR3-ANCA were evaluated (2 male, 3 female), mean age 34 (22-52) years. Pulmonary nodules (median 4, range 2-6), with or without cavitation were present in all patients. RTX induced initial B cell depletion (GPA following prolonged B cell depletion.

  9. Orofacial granulomatosis affecting lip and gingiva in a 15-year-old patient: A rare case report

    Directory of Open Access Journals (Sweden)

    Monika Bansal

    2015-01-01

    Full Text Available Orofacial granulomatosis (OFG is a rare disorder affecting the orofacial region, and clinically characterized by diffuse, nontender, soft to firm, painless swelling restricted to one or both lips and intraoral sites such as tongue, gingiva and buccal mucosa. Histologically, OFG is characterized by noncaseating granulomatous inflammation. The early diagnosis of OFG is essential for the better prognosis of the lesion. Delay in diagnosis of OFG results into formation of indurated and permanent swelling of the lip that not only compromises esthetic appearance but also causes impairment in function such as speaking and eating. Early diagnosis of OFG is challenging to the health care professionals due to clinical and histological resemblance to other chronic granulomatous disorders. Thus, dentists may act as a first person to diagnose the lesion and play an important role in the multidisciplinary treatment of granulomatous disorders. Here, we present a case of OFG affecting lips and gingiva in a 15-year-old patient without any identifiable systemic or local causes.

  10. Diffuse alveolar hemorrhage emerging one week after starting high-dose corticosteroid therapy for granulomatosis with polyangiitis (GPA) with systemic lupus erythematosus (SLE).

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kawakami, Atsushi

    2015-01-01

    A 69-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) based on the presence of skin granuloma, refractory otitis media, renal insufficiency and myeloperoxidase-antineutrophil cytoplasmic antibody positivity and slight lung opacity. He was treated with high-dose corticosteroid therapy. Despite the initial improvement of his renal function and a decrease in his C-reactive protein level, he suffered from an alveolar hemorrhage one week after the start of corticosteroid therapy. An anti-dsDNA antibody test was positive and the patient had hypocomplementemia. Elements of both GPA and systemic lupus erythematosus were thought to have affected his clinical course.

  11. Cardiac tamponade leading to the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature.

    Science.gov (United States)

    Yano, Toshiyuki; Ishimura, Shutaro; Furukawa, Tetsuaki; Koyama, Masayuki; Tanaka, Marenao; Shimoshige, Shinya; Hashimoto, Akiyoshi; Miura, Tetsuji

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA), which was previously called Churg-Strauss syndrome, is a necrotizing systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Cardiovascular complications, including eosinophilic myocarditis, are a major cause of mortality in this disorder. Acute pericarditis with slight pericardial effusion is a typical manifestation in EGPA, though hemodynamically significant pericardial effusion has been reported in a few cases. We report a case that initially presented with isolated cardiac tamponade, which was followed by systemic manifestations of EGPA over 3 weeks. Including the present case, previous EGPA cases with cardiac tamponade are reviewed to delineate its clinical characteristics.

  12. Accelerated atherosclerosis in patients with systemic autoimmune diseases

    NARCIS (Netherlands)

    De Leeuw, K.; Kallenberg, Cees; Bijl, Marc; Shoenfeld, Y.; Gershwin, M.E.; Shoenfeld, Y; Gershwin, ME

    2005-01-01

    Systemic autoimmune diseases such as systemic lupus erythematosus and Wegener's granulomatosis are associated with a significantly increased prevalence of cardiovascular disease (CVD) compared with age- and sex-matched controls. Many risk factors are involved in the pathogenesis of atherosclerosis,

  13. TNF-alpha bioactivity-inhibiting therapy in ANCA-associated vasculitis : Clinical and experimental considerations

    NARCIS (Netherlands)

    Huugen, Dennis; Tervaert, Jan Willem Cohen; Heeringa, Peter

    2006-01-01

    Wegener's granulomatosis, microscopic polyangiitis, idiopathic necrotizing crescentic glomerulonephritis, and Churg-Strauss syndrome are associated with the presence of ANCA with specificity for myeloperoxidase or proteinase 3. Current therapy consists mainly of corticosteroids and cyclophosphamide,

  14. Pathogenesis of PR3-ANCA associated vasculitis

    NARCIS (Netherlands)

    Kallenberg, C. G. M.

    2008-01-01

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

  15. Pauci-immune necrotizing glomerulonephritis

    NARCIS (Netherlands)

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

    2010-01-01

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

  16. Vasculitis Illness Perception (VIP) Study

    Science.gov (United States)

    2014-07-11

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

  17. 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...

  18. Brief Report

    DEFF Research Database (Denmark)

    Faurschou, Mikkel; Westman, Kerstin; Rasmussen, Niels;

    2012-01-01

    The NORAM (Nonrenal Wegener's Granulomatosis Treated Alternatively with Methotrexate [MTX]) trial demonstrated that MTX can replace cyclophosphamide (CYC) as remission-inducing treatment for patients with newly diagnosed early systemic antineutrophil cytoplasmic antibody-associated vasculitis...

  19. 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

  20. Pathogenesis of PR3-ANCA associated vasculitis

    NARCIS (Netherlands)

    Kallenberg, C. G. M.

    2008-01-01

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

  1. Antiproteinase 3-and antimyeloperoxidase-associated vasculitis

    NARCIS (Netherlands)

    Franssen, CFM; Stegeman, CA; Kallenberg, CGM; Gans, ROB; de Jong, PE; Hoorntje, SJ; Tervaert, JWC

    Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led

  2. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice

    NARCIS (Netherlands)

    Xiao, H; Heeringa, P; Hu, PQ; Liu, Z; Zhao, ML; Aratani, Y; Maeda, N; Falk, RJ; Jennette, JC

    2002-01-01

    Antineutrophil cytoplasmic autoantibodies (ANCAs) are identified in the circulation of approximately 80% of patients with pauci-immune necrotizing and crescentic glomerulonephritis and systemic small vessel vasculitis, such as microscopic polyangiitis and Wegener granulomatosis. The most common

  3. 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

    2011-01-01

    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 T

  4. Bilateral orbital cavernous haemangiomas.

    OpenAIRE

    Fries, P D; Char, D. H.

    1988-01-01

    Simultaneous bilateral orbital lesions are rare. The differential diagnosis includes orbital pseudotumour, metastasis, leukaemia, lymphoma, Wegener's granulomatosis, and neurofibromatosis. We report what we believe to be the first case of bilateral orbital cavernous haemangiomas.

  5. Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis

    NARCIS (Netherlands)

    Kallenberg, Cees G. M.

    2007-01-01

    Purpose of reviews This review focuses on recent advance in the diagnosis pathogenesis and treatment of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Recent findings Antineutrophil cytoplasmic autoantibodies are closely associated with Wegener's granulomatosis and micro

  6. Increased frequency of circulating IL-21 producing Th-cells in patients with granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Abdulahad, Wayel H; Lepse, Nikola; Stegeman, Coen A; Huitema, Minke G; Doornbos-van der Meer, Berber; Tadema, Henko; Rutgers, Abraham; Limburg, Pieter C; Kallenberg, Cees G M; Heeringa, Peter

    2013-01-01

    The present study aimed to explore a possible role for IL-21 producing Th-cells in the immunopathogenesis of granulomatosis with polyangiitis (GPA). Peripheral blood from 42 GPA patients in remission and 29 age-matched healthy controls (HCs) were stimulated in vitro, and the frequencies of IL-21 producing Th-cells were determined by flow cytometry. Since Th17-cells produce a low level of IL-21, IL-17 was also included in the analysis. Given that IL-21 is a hallmark cytokine for T follicular helper cells (T(FH)), we next evaluated the expression of their key transcription factor BCL-6 by RT-PCR and flow cytometry. To investigate the effect of IL-21 on autoantibody-production, PBMCs from GPA patients were stimulated in vitro with BAFF/IL-21 and total IgG and ANCA levels were measured in supernatants. In addition, the expression of IL-21-receptor on B-cells was analyzed. Percentages of IL-21 producing Th-cells were significantly elevated in GPA-patients compared to HCs, and were restricted to ANCA-positive patients. The expression of BCL-6 was significantly higher in ANCA-positive GPA-patients, as compared with ANCA-negative patients and HCs. IL-21 enhanced the production of IgG and ANCA in vitro in stimulated PBMCs from GPA patients. No difference was found in the expression of the IL-21-receptor on B-cells between ANCA-negative patients, ANCA-positive patients, and HCs. The increased frequency of circulating IL-21 producing Th-cells in ANCA-positive GPA patients and the stimulating capacity of IL-21 on ANCA-production suggest a role for these cells in the immunopathogenesis of GPA. Blockade of IL-21 could constitute a new therapeutic strategy for GPA.

  7. 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....

  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 or infla......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. [Biological treatment of rare inflammatory rheumatic diseases

    DEFF Research Database (Denmark)

    Baslund, B.

    2008-01-01

    The current status of the use of biological medicine in the treatment of adult onset morbus still, Wegeners granulomatosis and systemic lupus erythematosus (SLE) is reviewed. The need for controlled trials is emphasized. Anti-CD20 treatment for SLE patients with kidney involvement and patients wi...... with Wegeners granulomatosis seems promising. Anti-TNF and IL1 receptor antagonist can control disease activity in most patients with adult morbus still Udgivelsesdato: 2008/6/9...

  10. Serum, liver, and lung levels of the major extracellular matrix components at the early stage of BCG-induced granulomatosis depending on the infection route.

    Science.gov (United States)

    Kim, L B; Shkurupy, V A; Putyatina, A N

    2015-01-01

    Experiments on the model of mouse BCG-induced granulomatous showed that the content of glycosaminoglycans and proteoglycans in the extracellular matrix of the liver and lungs are changed at the early stages of inflammation (days 3 and 30 postinfection) before cell destruction in the organs begins. This is related to degradation of extracellular matrix structures. Their high content in the blood and interstitium probably contributes to the formation of granulomas, fibroblast proliferation and organ fibrosis. These processes depend on the infection route that determines different conditions for generalization of the inflammation process. Intravenous method of vaccine injection is preferable to use when designing the experiments simulating tuberculosis granulomatosis, especially for the analysis of its early stages.

  11. Poliangeitis amb Granulomatosis eosinofílica i asma al·lèrgica; dues monedes amb una mateixa cara?

    OpenAIRE

    Ramentol Sintas, Marc

    2013-01-01

    La síndrome de Churg-Strauss o Poliangeitis amb Granulomatosis eosinofílica (EGPA), clàssicament s'ha vinculat a un substrat asmàtic d'origen al·lèrgic. No obstant, l'evidència en aquest sentit és equívoca. L'objectiu d'aquest treball és identificar diferències clíniques i serològiques entre pacients amb EGPA i pacients asmàtics al·lèrgics, i determinar el perfil al·lèrgic d'un grup de pacients diagnosticats d'EGPA. Es comparen dos grups de pacients: un grup de diagnosticats d'EGPA, i un altr...

  12. Representation of Arctic mixed-phase clouds and the Wegener-Bergeron-Findeisen process in climate models: Perspectives from a cloud-resolving study

    Science.gov (United States)

    Fan, Jiwen; Ghan, Steven; Ovchinnikov, Mikhail; Liu, Xiaohong; Rasch, Philip J.; Korolev, Alexei

    2011-01-01

    Two types of Arctic mixed-phase clouds observed during the ISDAC and M-PACE field campaigns are simulated using a 3-dimensional cloud-resolving model (CRM) with size-resolved cloud microphysics. The modeled cloud properties agree reasonably well with aircraft measurements and surface-based retrievals. Cloud properties such as the probability density function (PDF) of vertical velocity (w), cloud liquid and ice, regimes of cloud particle growth, including the Wegener-Bergeron-Findeisen (WBF) process, and the relationships among properties/processes in mixed-phase clouds are examined to gain insights for improving their representation in General Circulation Models (GCMs). The PDF of the simulated w is well represented by a Gaussian function, validating, at least for arctic clouds, the subgrid treatment used in GCMs. The PDFs of liquid and ice water contents can be approximated by Gamma functions, and a Gaussian function can describe the total water distribution, but a fixed variance assumption should be avoided in both cases. The CRM results support the assumption frequently used in GCMs that mixed phase clouds maintain water vapor near liquid saturation. Thus, ice continues to grow throughout the stratiform cloud but the WBF process occurs in about 50% of cloud volume where liquid and ice co-exist, predominantly in downdrafts. In updrafts, liquid and ice particles grow simultaneously. The relationship between the ice depositional growth rate and cloud ice strongly depends on the capacitance of ice particles. The simplified size-independent capacitance of ice particles used in GCMs could lead to large deviations in ice depositional growth.

  13. Evaluation of automated multi-parametric indirect immunofluorescence assays to detect anti-neutrophil cytoplasmic antibodies (ANCA) in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

    Science.gov (United States)

    Csernok, Elena; Damoiseaux, Jan; Rasmussen, Niels; Hellmich, Bernhard; van Paassen, Pieter; Vermeersch, Pieter; Blockmans, Daniel; Cohen Tervaert, Jan-Willem; Bossuyt, Xavier

    2016-07-01

    The aim of this multicenter EUVAS study was to evaluate the diagnostic performance of multi-parametric indirect immunofluorescence (IIF) assays to detect anti-neutrophil cytoplasmic antibodies (ANCA) in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The study included 912 samples from diseased controls and 249 diagnostic samples from GPA (n=183) and MPA (n=66) patients. The performance of two automated multi-parametric assays [Aklides (Medipan/Generic Assays) and EuroPattern (Euroimmun)] combining IIF on cellular and purified antigen substrates was compared with two manual IIF analyses and with commercially available ELISAs for MPO- and PR3-ANCA (Euroimmun). The area under the curve (AUC) of the receiver operating characteristics (ROC) curve to discriminate AAV from controls was 0.925, 0.848, 0.855 and 0.904 for, respectively, the two manual analyses, Aklides and EuroPattern, and 0.959, 0.921 and 0.886 for, respectively, antigen-specific ELISA, antigen-coated beads, and microdot, respectively. Variation in pattern assignment between IIF methods was observed. The performance of IIF depends on the substrate used and the definition of IIF patterns. The performance of automated IIF is improved by multi-parameter testing (combined IIF and antigen-specific testing). Given the variability between IIF methods, the diagnostic importance of this technique is questioned. Copyright © 2016. Published by Elsevier B.V.

  14. Eosinophilic Granulomatosis With Polyangiitis With Thrombotic Microangiopathy: Is Simultaneous Systemic Lupus Erythematosus Associated With Clinical Manifestations?: A Case Report and Review of the Literature.

    Science.gov (United States)

    Fukui, Shoichi; Iwamoto, Naoki; Tsuji, Sosuke; Umeda, Masataka; Nishino, Ayako; Nakashima, Yoshikazu; Suzuki, Takahisa; Horai, Yoshiro; Koga, Tomohiro; Kawashiri, Shin-Ya; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kinoshita, Naóe; Kawakami, Atsushi

    2015-11-01

    Eosinophilic granulomatosis with polyangiitis (EGPA) is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which is characterized by vasculitis of the small to medium-sized vessels. On the contrary, thrombotic microangiopathy (TMA) is a life-threatening condition which can cause ischemic organ injury. Although several case reports have described patients with TMA associated with ANCA-associated vasculitis except for EGPA, there are no previous case reports of EGPA associated with TMA.A 71-year-old Japanese man was diagnosed with EGPA based on his asthma, eosinophilia, lung opacity, refractory sinusitis, and positive myeloperoxidase-ANCA. He was also diagnosed with TMA based on peripheral schizocytes and hemolytic anemia. We performed plasmapheresis and started high-dose corticosteroid therapy; thereafter, he improved promptly. His case also fulfilled the classification criteria of systemic lupus erythematosus (SLE) based on the pleural effusion, renal disorder, anemia, thrombocytopenia, positive antidouble-stranded DNA antibody, and low complement. Elements of SLE were thought to affect his clinical course.We reviewed 11 patients with EGPA or hypereosinophilic syndrome (HES) associated with SLE, including our case. Patients with EGPA or HES associated with SLE had more heart complications than patients with simple EGPA or simple HES did. Patients with EGPA or HES associated with SLE had more pleural effusion than patients with simple SLE did.Clinical manifestations of eosinophilia with SLE or SLE with eosinophilia may differ from simple SLE or simple eosinophilia.

  15. Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease

    Directory of Open Access Journals (Sweden)

    Sing Yun Chang

    2012-01-01

    Full Text Available Granulomatosis with polyangiitis (Wegener’s (GPA may mimic IgG4-related disease (IgG4-RD on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31% biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio. The IgG4+ cells and IgG4+/IgG+ ratio ranged 37–137/hpf and 44–83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal (n=4 or orbital/periorbital (n=4 sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.

  16. Unfavorably Altered Fibrin Clot Properties in Patients with Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome: Association with Thrombin Generation and Eosinophilia.

    Directory of Open Access Journals (Sweden)

    Lucyna Mastalerz

    Full Text Available Given reports on the increased prevalence of thromboembolic incidents in patients with eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome, we investigated whether fibrin clot properties are unfavorably altered in EGPA.Ex vivo plasma fibrin clot characteristics, including clot permeability, turbidimetry and efficiency of fibrinolysis using two assays, were investigated in 34 consecutive patients with remission in EGPA according to the Birmingham Vasculitis Activity Score version 3 (23 female, 11 male, aged 48 (range, 21-80 years. The control group comprised 34 age- and sex- matched volunteers.Compared with controls, patients with EGPA were characterized by denser fiber clots (estimated pore size, Ks, 7.30±0.93 vs 10.14±1.07 10-9 cm2, faster fibrin polymerization (lag phase in a turbidimetric curve, 41.8±3.6 vs 47.4±2.9 s, thicker fibrin fibers (maximum absorbance, ΔAbs, 0.87±0.09 vs 0.72±0.07, higher maximum levels of D-dimer released from clots (DDmax 4.10±0.46 vs 3.54±0.35 mg/L, and prolonged clot lysis time (t50%; 9.50±1.45 vs 7.56±0.87 min; all p<0.0001. Scanning electron microscopy images confirmed denser plasma fibrin networks composed of thinner fibers formed in EGPA. Antineutrophil cytoplasmic antibody status and C-reactive protein did not affect clot variables. Multivariate analysis adjusted for fibrinogen showed that Ks was predicted by eosinophil count, peak thrombin generation, factor VIII, and soluble CD40 ligand, whereas eosinophil count, peak thrombin generation and antiplasmin predicted t50%.This study is the first to show that EGPA is associated with prothrombotic plasma fibrin clot phenotype, which may contribute to thromboembolic manifestations reported in this disease.

  17. Higher antineutrophil cytoplasmic antibody (C-ANCA) titers are associated with increased overall healthcare use in patients with sinonasal manifestations of granulomatosis with polyangiitis (GPA).

    Science.gov (United States)

    Janisiewicz, Agnieszka M; Klau, Marc H; Keschner, David B; Lehmer, Randy R; Venkat, Kumar V; Medhekar, Swati S; Chang, Parke K; Badran, Karam; Leary, Ryan; Garg, Rohit; Nguyen, Andrew A; Lee, Jivianne T

    2015-01-01

    Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous airway inflammation and vasculitis. Sinonasal involvement occurs in more than 80% cases, with antineutrophil cytoplasmic antibody (C-ANCA) titers used as a marker of disease severity. The purpose of this study was to determine whether C-ANCA levels impact radiographic findings and healthcare use in patients with sinonasal GPA. A retrospective review was performed on GPA patients evaluated in a multidisciplinary rheumatologic/otolaryngologic clinic from 2008 to 2013. Data were collected with respect to age, gender, clinical presentation, C-ANCA titers, Lund-Mackay (LM) scores, surgical interventions, and healthcare use, the latter of which were determined by assessing the number of rheumatology/otolaryngology clinic visits, computed tomography (CT) scans, and email/telephone encounters. A total of 44 patients were identified, 11 male and 33 female. Sinonasal manifestations were evident in 70.4%, with chronic rhinosinusitis (CRS) (41.9%), septal perforation (38.7%), and crusting (32.2%) the most common findings. No significant differences in number of CT scans (p = 0.10) or mean LM scores (p = 0.47) were found between patients with more than or equal to 1:80 and less than 1:80 C-ANCA titers, respectively. However, overall healthcare use was increased in the more than or equal to 1:80 C-ANCA group (n = 28) compared with less than 1:80 (n = 16), with a significantly greater number of rheumatologic/otolaryngologic encounters (mean 121 versus 69.2, p = 0.03) noted. When otolaryngologic healthcare use was specifically examined, the average number of encounters was also higher in more than or equal to 1:80 C-ANCA patients (31.9 versus 22.9), but this difference was not statistically significant (p = 0.16). Sinonasal GPA patients with presenting C-ANCA titers more than or equal to 1:80 demonstrated significantly greater overall healthcare use than their lower C

  18. Necrotizing RPGN with linear anti IgG deposits in a patient with history of granulomatosis with polyangiitis: a case report

    Directory of Open Access Journals (Sweden)

    Parekh N

    2014-11-01

    Full Text Available Ninad Parekh, Edward Epstein, Suzanne El-Sayegh Department of Medicine, Division of Nephrology, Staten Island University Hospital, Staten Island, NY, USA Introduction: Diagnosing the etiology of a rapidly progressive glomerulonephritis is of vital importance to guide appropriate therapeutic management. This case highlights the complexity involved in establishing diagnosis when presentation is atypical. In certain cases diagnosis cannot be established based on clinical presentation or biopsy findings alone, and critical analysis of biopsy findings in context of clinical presentation is crucial to guide the clinical decision-making process.Case presentation: A 47-year-old Hispanic male with history of granulomatosis with polyangiitis (GPA in remission on azathioprine, presented with fatigue and lethargy. Physical examination was unremarkable. Laboratory data revealed elevated creatinine and otherwise normal electrolytes. Urinalysis showed numerous dysmorphic red blood cells with few red cell casts. His serologic results were all negative except anti-proteinase-3 antibody at very low titers. Kidney biopsy showed necrotizing crescentic glomerulonephritis with linear immunoglobulin G staining along the basement membrane.Conclusion: This case presented conflicting serologic and histopathologic findings. The presence of anti-proteinase-3 antibody supported diagnosis of recurrence of GPA. However, linear staining of immunoglobulin G (IgG on immunofluorescence (IF staining of renal biopsy supported anti-glomerular basement membrane (GBM disease. The treatment of anti-GBM disease and GPA both involve immunosuppression with prednisone and cyclophosphamide. However, patients with anti-GBM disease are also treated with plasmapheresis early in the disease presentation to prevent further damage. The patient with GPA, on the other hand, was shown to benefit from plasmapheresis only in the case of severe renal disease (serum creatinine level more than 5 mg/dL or

  19. Fluoxetin-induced pulmonary granulomatosis.

    Science.gov (United States)

    de Kerviler, E; Trédaniel, J; Revlon, G; Groussard, O; Zalcman, G; Ortoli, J M; Espié, M; Hirsch, A; Frija, J

    1996-03-01

    A patient treated with fluoxetin for a manic depressive disorder developed pulmonary inflammatory nodules with noncaseating giant cell granulomas, interstitial pneumonia and non-necrotizing vasculitis, whilst remaining asymptomatic. A progressive resolution of pulmonary nodules occurred after withdrawal of the offending agent, and the chest radiograph returned to normal in 9 months. The diagnosis was assessed by an open lung biopsy.

  20. Pulmonary vasculitis.

    Science.gov (United States)

    Lally, Lindsay; Spiera, Robert F

    2015-05-01

    Pulmonary vasculitis encompasses inflammation in the pulmonary vasculature with involved vessels varying in caliber from large elastic arteries to capillaries. Small pulmonary capillaries are the vessels most commonly involved in vasculitis affecting the lung. The antineutrophil cytoplasmic antibody-associated vasculitides, which include granulomatosis with polyangiitis (formerly Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), are the small vessel vasculitides in which pulmonary vasculitis is most frequently observed and are the major focus of this review. Vasculitic involvement of the large pulmonary vessels as may occur in Behçet syndrome and Takayasu arteritis is also discussed.

  1. A abordagem cognitiva da metáfora em perspectiva pragmática nos séculos dezoito e dezenove: as contribuições de Lambert e Wegener a discussões atuais

    Directory of Open Access Journals (Sweden)

    Ulrike Agathe Schröder

    2014-01-01

    Full Text Available Desde o surgimento da Teoria Conceptual da Metáfora, há mais de trinta anos, houve uma série de autores que apontaram a falta de contextualização historiográfica, uma vez que a maioria das hipóteses sobre a metáfora conceptual e até boa parte dos exemplos já são antecipadas desde o século dezessete em reflexões filosóficas, antropológicas, psicológicas e linguísticas. O presente trabalho pretende apresentar dois pensadores dos séculos dezoito e dezenove, a saber, o filósofo Johann Heinrich Lambert (1965 e o linguista Philipp Wegener (1991, que não apenas já desenvolvem uma teoria cognitiva da metáfora cotidiana, como também já conseguem integrar aspectos pragmáticos, que atualmente são redescobertos como fatores descuidados pela primeira geração da Linguística Cognitiva. Sendo assim, as duas concepções podem lançar uma ponte a discussões atuais, uma vez que ambas conseguem apontar caminhos para superar tanto o abismo entre o sujeito solipsista e a sociedade, como a dicotomia entre universalismo e relatividade cultural. Os dois autores entendem a metáfora como fenômeno cognitivo e, ao mesmo tempo, intersubjetivo, por ser negociado entre os participantes em situações comunicativas concretas.

  2. Clinical features and diagnosis of primary cerebral lymphomatoid granulomatosis%原发性颅内淋巴瘤样肉芽肿病的临床特点及诊断

    Institute of Scientific and Technical Information of China (English)

    卫华; 李存江; 朴月善; 卢德宏; 周静安

    2014-01-01

    目的:总结原发性颅内淋巴瘤样肉芽肿病的临床特点、影像学及组织病理学特点。方法报告我院收治的1例原发性颅内淋巴瘤样肉芽肿病病例,并对相关文献进行复习。结果患者,女,28岁,癫痫发作3年余,头颅MRI显示散在多发病灶,环形或结节状强化,病灶周围水肿明显。除颅内多发病灶外其他器官未受累。脑组织活检病理学提示淋巴瘤样肉芽肿病。结论原发性颅内淋巴瘤样肉芽肿病影像学有一定特点,但无特异性,因此颅内占位性病变或散在、浸润性病变患者需注意和本病鉴别。单纯颅内淋巴瘤样肉芽肿病预后较好。%Objective To report a case of primary brain lymphomatoid granulomatosis (LYG), and summarize its imaging, histopathological characteristics and prognosis. Methods The clinical, pathological, imaging and laboratory data of the patient were presented, and the pertinent literature were reviewed to discuss the importance of this rare entity. Re-sults A 28-year-old woman had been suffering from epilepsia for more than 3 years. MRI revealed multiple focal in-tra-parenchymal lesions, with heterogeneous enhancement surrounded by perilesional edema in the left frontal and tempo-ral lobe. The focal resection of the lesion in the left frontal was performed and lymphomatoid granulomatosis was diagnosed based on the histopathological examination. Conclusion CNS-LYG is a rare disease that should be considered in the dif-ferential diagnosis of both diffuse and space-occupying cerebral lesions. Primary cerebral LYG appears to have a better prognosis than systemic LYG with CNS localization.

  3. An Unusual Radiologic Manifestation of Pulmonary Tuberculosis with Bilateral Multiple Lung Nodules and Diffuse Alveolar Hemorrhage: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Jeong, Seo In; Seon, Hyun Ju; Kim, Yun Hyeon [Dept. of Radiology, Chunnam National University Hospital, Gwangju (Korea, Republic of); Choi, Sung [Dept. of Radiology, Chunnam National University Hwasun Hospital, Hwasun(Korea, Republic of)

    2011-12-15

    Pulmonary tuberculosis presenting as bilateral multiple lung nodules or diffuse alveolar hemorrhage is very rare. Here, we report a case of pulmonary tuberculosis presenting as bilateral multiple lung nodules and diffuse alveolar hemorrhage mimicking granulomatous vasculitis, such as Wegener's granulomatosis.

  4. Pathogenesis of pulmonary vasculitis

    NARCIS (Netherlands)

    Heeringa, P; Schreiber, A; Falk, RJ; Jennette, JC

    2004-01-01

    Vasculitis is inflammation of blood vessels and can affect any type of vessel in any organ. Pulmonary vasculitis usually is a component of a systemic small vessel vasculitis. Three major forms of small vessel vasculitis that often affect the lungs are Wegener's granulomatosis, microscopic polyangiit

  5. Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases

    Science.gov (United States)

    2005-06-23

    Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

  6. The value of serial ANCA testing during follow-up studies in patients with ANCA-associated vasculitides. A review

    NARCIS (Netherlands)

    Tervaert, JWC

    1996-01-01

    Anti-neutrophil cytoplasmic autoantibodies (ANCA) have specificity for cytoplasmic proteins of myeloid cells. Many antigens recognized by ANCA have already been described. In most patients with primary forms of vasculitis such as Wegener's granulomatosis (WG), the Churg-Strauss Syndrome (CSS) and mi

  7. Pathogenesis of pulmonary vasculitis

    NARCIS (Netherlands)

    Heeringa, P; Schreiber, A; Falk, RJ; Jennette, JC

    2004-01-01

    Vasculitis is inflammation of blood vessels and can affect any type of vessel in any organ. Pulmonary vasculitis usually is a component of a systemic small vessel vasculitis. Three major forms of small vessel vasculitis that often affect the lungs are Wegener's granulomatosis, microscopic

  8. Prognosis of microscopic polyangiitis with renal involvement: report of 60 Chinese patients

    Institute of Scientific and Technical Information of China (English)

    HU Wei-xin; LIU Zhi-hong; LIU Chun-bei; TANG Zheng; WANG Qing-wen; CHEN Hui-ping; LI Lei-shi

    2005-01-01

    @@ With the widespread clinical application of renal biopsy and serum antineutrophil cytoplasmic antibody (ANCA) testing, the percentage of renal injuries caused by systemic small vessel vasculitis (SVV) including microscopic polyangiitis (MPA)and Wegener's granulomatosis (WG) is on the rise in China.

  9. Granuloma formation in ANCA-associated vasculitides

    NARCIS (Netherlands)

    Lamprecht, Peter; Wieczorek, Stefan; Epplen, Jorg T.; Ambrosch, Petra; Kallenberg, Cees G. M.

    2009-01-01

    Granuloma formation is a key pathologic finding in two of the anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides: Wegener's granulomatosis (WG) and Churg-Strauss syndrome (CSS). So far, no animal models have been established convincingly reproducing both vasculitic and granuloma

  10. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature.

    Science.gov (United States)

    von Vigier, R O; Trummler, S A; Laux-End, R; Sauvain, M J; Truttmann, A C; Bianchetti, M G

    2000-05-01

    In adults, the term specific pulmonary renal syndrome describes disorders with pulmonary and glomerular manifestations and includes Wegener's granulomatosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating glomerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we reviewed the charts of 21 pediatric patients with pulmonary renal syndromes treated by the Department of Pediatrics, University of Bern between 1991 and 1998; we also reviewed the pediatric literature that deals with specific pulmonary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vasculitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1) and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thromboembolism (N = 2), and pulmonary infection (N = 1) complicating the course of a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of specific pulmonary renal syndrome other than systemic lupus erythematosus: Wegener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Schönlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childhood. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lupus erythematosus, Henoch-Schönlein purpura and hemolytic-uremic syndrome occasionally have both pulmonary and renal features. Copyright 2000 Wiley-Liss, Inc.

  11. Correlação anátomo-clínica: granulomatose sistêmica por talco em drogadicto hiv negativo Systemic talc granulomatosis in a HIV-negative intravenous drug addict

    Directory of Open Access Journals (Sweden)

    D.F. da Cunha

    1999-07-01

    Full Text Available É descrita a correlação anatomopatológica de um paciente usuário de drogas injetáveis com talcose sistêmica acometendo pulmões, pleuras, fígado, baço e linfonodos e que desenvolveu hipertensão arterial pulmonar e cor pulmonale. Destaca-se o fato de que apesar do uso crônico de drogas injetáveis e outros comportamentos de alto risco para infecção pelo retrovírus, o paciente era HIV negativo, não apresentava linfocitopenia, nem alterações histopatológicas compatíveis com AIDS à necropsia.Anatomo-pathological correlation in a case of systemic talc granulomatosis affecting lungs, pleura, liver, spleen and mesenteric lymph nodes resulting in pulmonary arterial hypertension and cor pulmonale is described. The patient, a 26-year-old male HIV-negative intravenous drug addict had no lymphopenia or any histopathologic findings at necroscopy compatible with AIDS, despite of a chronic high-risk behavior favoring this illness.

  12. Peritoneal Keratin Granulomatosis Associated with Endometrioid Adenocarcinoma of the Uterine Corpus in a Woman with Polycystic Ovaries: A Potential Pitfall—A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Helen J. Trihia

    2017-01-01

    Full Text Available Peritoneal keratin granulomatosis is a rare condition included under granulomatous lesions of the peritoneum. It can be secondary to neoplasms of the female genital tract and can mimic carcinomatosis intraoperatively. A case of a 40-year-old woman with a history of polycystic ovaries and a chief complaint of vaginal bleeding is presented. She was diagnosed with endometrioid adenocarcinoma with squamous differentiation in endometrial curettings. Intraoperatively, many peritoneal nodules were found, interpreted as peritoneal carcinomatosis. The woman underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy, bilateral pelvic lymphadenectomy, and appendicectomy. Multiple biopsies were taken, as well as peritoneal washings. Microscopic examination revealed multiple keratin granulomas on the serosal surface of the ovaries, fallopian tubes, appendix, and omentum. Lymph node metastasis was not found. Peritoneal keratin granulomas (PKGs have been reported in cases of endometrioid adenocarcinoma with squamous differentiation of the uterine corpus, ovary, and atypical adenomyoma. It should be noted that the prognosis of cases of peritoneal keratin granulomas without viable tumor cells is favourable and that the histologic examination is essential for its diagnosis. We report a case of PKG in a patient with endometrial carcinoma with squamous differentiation, being the first in a woman with polycystic ovaries.

  13. Sociale media: nieuwe wegen naar sociale innovatie

    NARCIS (Netherlands)

    Salverda, I.E.; Jagt, van der P.D.; Willemse, R.; Onwezen, M.C.; Top, J.L.

    2013-01-01

    Hoewel de rol en impact van internet en de sociale media in de samenleving algemeen worden aangenomen, is het nog niet duidelijk of en hoe het communiceren en delen van informatie via internet en de sociale media bijdragen aan het ontstaan van sociale innovatie. Hoofdvragen van deze verkenning zijn

  14. Stijging van het aantal operaties wegens stressincontinentie

    NARCIS (Netherlands)

    Vierhout, M.E.

    2005-01-01

    Since the introduction of the minimally invasive tension-free vaginal tape (TVT) the number of operations performed for treatment of stress urinary incontinence has increased dramatically from over 1600 in 1999 to more than 4200 in 2003. Both gynaecologists and urologists now perform more TVTs and f

  15. Pulmonary lymphomatoid granulomatosis: an immunohistochemical and gene rearrangement study%肺淋巴瘤样肉芽肿病的免疫表型及基因重排研究

    Institute of Scientific and Technical Information of China (English)

    冯瑞娥; 刘鸿瑞; 刘彤华; 陈杰; 凌庆; 师晓华; 钟定荣; 罗玉风; 曹金伶

    2011-01-01

    目的 观察肺淋巴瘤样肉芽肿病的细胞组成、免疫表型和分子生物学改变.方法 回顾性分析北京协和医院9例肺淋巴瘤样肉芽肿病患者的临床病理情况.其中5例为开胸肺活检标本,3例为肺叶切除标本,1例尸检.标本经4%甲醛固定,石蜡包埋,常规切片,HE染色.免疫组织化学EnVision法染色(抗体包括CD20、CD3、CD56),原位杂交检测EB病毒,采用聚合酶链反应进行Ig和TCR基因重排检测.结果 9例肺淋巴瘤样肉芽肿患者,年龄3~59岁,男∶女=3:6.9例患者肺组织内病变分布均显示以血管为中心的淋巴细胞浸润为特点.免疫组织化学显示以CD3阳性的T淋巴细胞占绝对优势,散在不等数量的CD20阳性的B细胞,CD56均为阴性.8例行EB病毒原位杂交,4例阳性细胞数20%,1例为15%.按照WHO的3级分级方法,Ⅰ级为4例,Ⅱ级1例,Ⅲ级4例.6例行基因重排检测,3例显示有Ig基因重排阳性,其中,1例为Ⅱ级病变,2例为Ⅲ级病变.6例TCR重排检测均为阴性.随访时间0.5~6.5年不等,9例患者中3例死亡,2例存活,4例失访.结论 部分肺淋巴瘤样肉芽肿病,特别是Ⅱ、Ⅲ级患者,有B淋巴细胞克隆性增生,提示其为淋巴瘤性病变.%Objective To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis. Methods Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies. Results The Histologically, all cases showed lymphocytic infiltration surrounding the blood vessels and in the perivascular areas. Most of these lymphoid cells expressed T-cell marker CD3. There were also variable numbers of CD20-positive B cells. The staining for CD56 was negative. According to the WHO

  16. Ocular manifestations of granulomatosis with polyangiitis: an analysis of 164 patients%肉芽肿性多血管炎患者眼部表现临床分析

    Institute of Scientific and Technical Information of China (English)

    陈迪; 闵寒毅; 赵久良; 曾小峰

    2016-01-01

    Objective To review the ophthalmic features of granulomatosis with polyangiitis (GPA).Methods Clinical data of 164 GPA patients diagnosed in Peking Union Medical College Hospital during the past 10 years were retrospectively reviewed.Results Among 164 patients,48 (29.3%) had ocular involvement with a male to female ratio of 1.4:1 and an average age of (45 ± 15) years.Nine patients (5.5%) were first diagnosed at the ophthalmology department.Scleritis (15.9%,26/164),orbit lesions (13.4%,22/164) and peripheral corneal ulcer (9.1%,15/164) were the three most common ophthalmic complications.Twenty-two patients (13.4%) had positive findings in the orbit imaging.Six patients (3.7%,6/164) lost their vision due to GPA.Conclusion The ocular manifestations of GPA are diverse and complicated.GPA may affect every component of the eye,sclera,orbit and cornea are the most frequently affected sites of the eye.%目的 分析肉芽肿性多血管炎(GPA)眼部临床表现的特点.方法 回顾性分析2005年8月至2015年8月北京协和医院确诊的164例GPA患者的眼科临床资料.结果 164例患者中眼部受累者48例(29.3%),男女比例为1.4:1,平均年龄(45±15)岁;首诊于眼科者9例(5.5%);巩膜炎、眼眶病变和边缘性角膜溃疡是最常见的GPA眼部表现,分别为26例(15.9%)、22例(13.4%)、15例(9.1%);眼眶MRI检查阳性者22例(13.4%).GPA致盲率3.7% (6/164).结论 GPA眼部表现复杂多样,可侵犯眼部所有组织,巩膜、眼眶和角膜是其最常累及的部位.

  17. CASO -2016: Sindrome de Churg Strauss: a propósito de dos casos en el Hospital San Vicente de Paúl

    OpenAIRE

    Hu Liang, Huixian; Leitón Chaves, Andrés; Salas Mena, Claudio

    2016-01-01

    Las vasculitis asociadas a anticuerpos citoplasmá-ticos antineutrófilos, clasificadas como vasculitis de pequeños vasos incluyen: granulomatosis con poliangeítis (enfermedad de Wegener), poliangeítis microscópica y la granulomatosis eosinofilica con poliangeítis (GEPA) (Churg Strauss). La enferme-dad de Churg Strauss es una de las vasculitis necro-tizantes sistémicas más raras, pero potencialmente mortal. La incidencia internacional de la enferme-dad es de aproximadamente 2.5 casos por cada 1...

  18. Cavitary pulmonary nodules in atypical collagen disease and lupoid drug reaction. Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Muren, C.; Strandberg, O.

    The case histories of two patients with cavitary pulmonary nodules and the findings at chest radiography are reviewed. The first patient had a connective tissue disease with features common to systematic lupus erythematosus and Wegener's granulomatosis. In the second patient the lung changes developed as part of a drug reaction to carbamezapine and/or phenytoin. The common denominator of the cavitating nodules is probably the presence of granulomas, developing as a sequela of pulmonary vasculitis. (orig.).

  19. Bilateral scleritis and sclerokeratitis associated with IgA nephropathy

    OpenAIRE

    Garza-Leon, Manuel; Flores, Diana; Alarcón-Galván, Gabriela; Sánchez-Martínez, Concepción

    2012-01-01

    Purpose The purpose of this study is to report a case of bilateral nodular scleritis in a patient with final diagnosis of IgA nephropathy. Methods This is an observational case report. Results A male patient, 42 years old, presented with a bilateral nodular scleritis and OD sclerokeratitis. He had a previous history of acute otitis media and developed posterior renal failure and arterial hypertension. Clinical and systemic findings suggest Wegener's granulomatosis. A kidney biopsy was perform...

  20. [Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine].

    Science.gov (United States)

    Maisch, B

    2015-02-01

    In this article the diagnostics, differential diagnosis, laboratory findings, multimodal imaging and treatment of vasculitis of small, medium and large vessels as well as granulomatous and eosinophilic vascular diseases are described in the context of previous and current European League against Rheumatism (EULAR) and American College of Rheumatology (ACR) recommendations. Vasculitis is a syndrome which is part of various clinical disease entities, such as Wegener's granulomatosis, polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, cryoglobulinemia and other forms of vasculitis.

  1. [Vasculitis: New nomenclature of the Chapel Hill consensus conference 2012].

    Science.gov (United States)

    Holl-Ulrich, K

    2014-11-01

    Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis.

  2. Granulomatosis sarcoidea necrosante con compromiso sistémico Necrotizing sarcoid granulomatosis with systemic involvement

    Directory of Open Access Journals (Sweden)

    María Elena Ceballos

    2006-09-01

    Full Text Available Mujer mapuche de 70 años quien en el curso de 1 año presentó baja de peso, ojo rojo bilateral y úlcera corneal que evolucionó hacia la perforación con herniación uveal y pérdida de la visión. Posteriormente se agregaron lesiones nodulares, violáceas, sensibles, algunas ulceradas en las zonas distales de ambas extremidades inferiores. La TAC de tórax demostró múltiples nódulos pulmonares. Tras un estudio exhaustivo se descartaron la tuberculosis y otras infecciones. Tanto la biopsia de las lesiones cutáneas como la biopsia pulmonar obtenida mediante toracotomía confirmaron la presencia de granulomas necrotizantes no caseificantes y arteritis granulomatosa. Se trató con esteroides y ciclofosfamida con mejoría de su condición general y regresión de las lesiones cutáneas y pulmonares en menos de 1 mes. Se realizó un trasplante de cornea. A los seis meses de seguimiento continúa en tratamiento, no ha presentado recaídas y tiene recuperación parcial de la visiónA 70-year-old mapuche female presented with a 1 year history of weight loss, bilateral red painful eyes and corneal ulcer that evolved into perforation and uveal herniation with complete visual loss. Reddish and painful nodules appeared on the distal aspect of both lower extremities, that ulcerated after months. A thoracic CT scan showed multiple pulmonary nodules. Infectious diseases were ruled out. An open lung biopsy and a skin biopsy were performed and both showed non-caseating necrotizing granulomas and granulomatous arteritis. Steroids and cyclophosphamide were started with complete regression of skin and pulmonary lesions within a month. Corneal transplantation was done. After 6 months follow-up, the patient is on low-dose of steroids, free of disease with partial vision recovery

  3. Bouwmaterialen paalmatrassystemen: Delft Cluster Blijvend Vlakke Wegen, WP3

    NARCIS (Netherlands)

    Van der Meij, R.; Van Ruijven, J.

    2007-01-01

    Dit rapport betreft een literatuurstudie naar bouwmaterialen die in paalmatrassystemen worden gebruikt. De volgende aspecten komen aan bod: • Materialen voor paalmatrassystemen (palen, geotextielen, matrasmateriaal) • Hiaten in kennis (op basis van interviews) Door middel van deze literatuurstudie e

  4. Verkeersonveiligheid op rijkswegen en belangrijke 80 km/uur-wegen.

    NARCIS (Netherlands)

    Janssen, S.T.M.C.

    1993-01-01

    In this project, experience was gained with the collection, processing and presentation of data with respect to road hazard on so-called 'A' and 'N' roads (motorways and arterial rural roads). Parts of the A and N road networks in the Netherlands were charted for three provinces. A distinction was m

  5. Beknopte literatuurstudie inzake categorie-indeling van wegen.

    NARCIS (Netherlands)

    Dijkstra, A. & Twisk, D.A.M.

    1992-01-01

    This literature study describes the road categorization from the road user point of view. The study describes the following subjects: (1) the mental load of road users; (2) road categories in relation to road safety; (3) a model for the traffic and transport system; (4) recognition of road types; an

  6. Bruggen en wegen naar een duurzame Randstad in 2040

    NARCIS (Netherlands)

    d'Haese, N.

    2008-01-01

    Dit rapport werd geschreven door het Dutch Research Institute for Transitions in opdracht van Delft Cluster. Het rapport kadert binnen het PIZZA-project, een samenwerking tussen 6 Bsik-programma’s van de 8 voor ruimte en KSI. Het doel van dit project is een methodiek voor transitiemonitoring te ontw

  7. [Vasculitis. New nomenclature of the Chapel Hill consensus conference 2012].

    Science.gov (United States)

    Holl-Ulrich, K

    2013-11-01

    In recent years, many advances have been made in our understanding of vasculitis etiopathology as well as of different disease courses. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature reflects current knowledge about etiopathology, in addition to the descriptive principles of vessel size and type of inflammation. Anti-neutrophil cyptoplasmic antibody (ANCA)-associated vasculitides have been classified as a separate group, as opposed to immune complex small vessel vasculitis. In cases where consensus was achieved, eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes, focussing on those types that are relevant to the histopathologist, and summarizes the results of important new articles on morphology and clinical picture of vasculitis.

  8. Cardiovascular magnetic resonance for evaluation of heart involvement in ANCA-associated vasculitis. A luxury or a valuable diagnostic tool?

    Science.gov (United States)

    Mavrogeni, Sophie; Markousis-Mavrogenis, George; Kolovou, Genovefa

    2014-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-related vasculitis is a systemic small-vessel vasculitis, including 3 clinical syndromes: granulomatosis with polyangiitis, known as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and the Churg-Strauss syndrome (CSS). ANCA-related vasculitis usually presents with severe kidney or pulmonary disease, has a mortality of 28% at 5 years, and also contributes to increased morbidity in vasculitis patients. Cardiac involvement in this entity may have different forms, including coronary vessels, pericarditis, myocarditis, endocarditis, myocardial infarction and subendocardial vasculitis that can contribute to reduced life expectancy. Cardiovascular magnetic resonance using oedema and fibrosis imaging can early reveal, noninvasively and without radiation, heart involvement during vasculitis, undetected by other imaging techniques and guide further risk stratification and treatment of these patients.

  9. Diffuse abnormalities of the trachea: computed tomography findings

    Energy Technology Data Exchange (ETDEWEB)

    Marchiori, Edson [Universidade Federal Fluminense (UFF), Niteroi, RJ (Brazil). Dept. de Radiologia]. E-mail: edmarchiori@gmail.com; Pozes, Aline Serfaty [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Faculdade de Medicina. Dept. de Radiologia; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Escola de Medicina. Dept. de Radiologia; Escuissato, Dante Luiz [Universidade Federal do Parana (UFPR), Curitiba, PR (Brazil). Dept. de Radiologia; Irion, Klaus Loureiro [The Cardiothoracic Centre NHS Trust, Liverpool (United Kingdom); Araujo Neto, Cesar de [Universidade Federal da Bahia (UFBA), Salvador, BA (Brazil). Dept. de Radiologia; Barillo, Jorge Luiz; Zanetti, Glaucia [Faculdade de Medicina de Petropolis (FMP), RJ (Brazil). Dept. de Cirurgia Clinica; Souza, Carolina Althoff [University of Ottawa, Ottawa, ON (Canada)

    2008-01-15

    The aim of this pictorial essay was to present the main computed tomography findings seen in diffuse diseases of the trachea. The diseases studied included amyloidosis, tracheobronchopathia osteochondroplastica, tracheobronchomegaly, laryngotracheobronchial papillomatosis, lymphoma, neurofibromatosis, relapsing polychondritis, Wegener's granulomatosis, tuberculosis, paracoccidioidomycosis, and tracheobronchomalacia. The most common computed tomography finding was thickening of the walls of the trachea, with or without nodules, parietal calcifications, or involvement of the posterior wall. Although computed tomography allows the detection and characterization of diseases of the central airways, and the correlation with clinical data reduces the diagnostic possibilities, bronchoscopy with biopsy remains the most useful procedure for the diagnosis of diffuse lesions of the trachea. (author)

  10. Presenting A Case with Tubulointerstitial Nephritis and Uveitis (TINU- Syndrome

    Directory of Open Access Journals (Sweden)

    E Fotouhi Ardakani

    2008-10-01

    Full Text Available Concurrence of interstitial nephritis and uveitis named tubulointestitioal nephritis and uveitis syndrome (TINU are unusual and uncommon presentations of interstitial nephritis. This syndrome is considered after ruling out other differential diagnoses. A-38-year old man presented with acute renal failure and uveitis. The histologic findings of renal biopsy showed acute tubulointestitioal nephritis. The patient had no clinical and paraclinical manifestations of other etiologies of interstitial nephritis and uveitis such as Wegener's granulomatosis , Sjogren's syndrome or sarcoidosis. The diagnosis of TINU-Syndrome was therefore considered. The patient was treated by oral and ophthalmic prednisolone and had a good response to treatment.

  11. Etiologic diagnosis of jaw osteonecrosis, other than bisphosphonate and radiotherapy related osteonecrosis.

    Science.gov (United States)

    Magremanne, M; Picheca, S; Reychler, H

    2014-02-01

    Our purpose was to highlight the various etiologies of maxillo-mandibular osteonecrosis, other than radiotherapy and biphosphonate related osteitis that have been abundantly reported. We performed a PubMed search from August 1, 1972 to August 1, 2012 using the following MeSH terms: "osteonecrosis", "bone", "necrosis", "jaw", "maxilla", "mandible", "palate", "oral", "avascular necrosis", NOT "bisphosphonate" NOT "osteoradionecrosis". Most cases of osteonecrosis were iatrogenic. Viral, mycotic, or bacterial infections were less frequent causes. Cocaine abuse, Wegener's granulomatosis, and N/K lymphoma were other etiologies. It is important to identify the various etiologies rapidly to manage this sometimes very mutilating condition adequately.

  12. [Etiologic diagnosis of jaw osteonecrosis, other than bisphosphonate and radiotherapy related osteitis].

    Science.gov (United States)

    Magremanne, M; Picheca, S; Reychler, H

    2014-02-01

    Our purpose was to highlight the various etiologies of maxillo-mandibular osteonecrosis, other than radiotherapy and biphosphonate related osteitis that have been abundantly reported. We performed a PubMed search from August 1, 1972 to August 1, 2012 using the following MeSH terms: "osteonecrosis", "bone", "necrosis", "jaw", "maxilla", "mandible", "palate", "oral", "avascular necrosis", NOT "bisphosphonate" NOT "osteoradionecrosis". Most cases of osteonecrosis were iatrogenic. Viral, mycotic, or bacterial infections were less frequent causes. Cocaine abuse, Wegener's granulomatosis, and N/K lymphoma were other etiologies. It is important to identify the various etiologies rapidly to manage this sometimes very mutilating condition adequately.

  13. 原发于中枢神经系统的淋巴瘤样肉芽肿:病例报告并文献分析%A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis:case report and literature review

    Institute of Scientific and Technical Information of China (English)

    付永娟; 朴月善; 陈莉; 李存江; 卢德宏

    2012-01-01

    Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG). Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57-year-old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI) showed space-occupying lesions in bilateral frontal lobes, with TiWI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray-white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein-Barr virus (EBV) was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed.%目的 探讨原发于中枢神经系统的淋巴瘤样肉芽肿的临床表现、影像学及病理学特点.方法 回顾分析一例原发于中枢神经系统的淋巴瘤样肉芽肿患者的临床及影像学表现、组织学及分子生物学特点,并复习相关文献.结果 男性患者,57岁.表现为记忆力减退3个月,地点定向障碍,计算力和理解判断力下降.影像学检查显示双侧额叶占位性病变,呈等T1、长T2信号,强化不均匀.

  14. ANCA-negative limited Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Ghosh A

    2004-03-01

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

  15. Polishing surgical metal pieces, granulomatosis and mineralogical analysis.

    Science.gov (United States)

    Catinon, Mickaël; Chemarin, C; Roux, E; Cavalin, C; Rosental, P-A; Thivolet-Bejui, F; Vincent, M

    2016-08-01

    This report describes the case of a 44-year-old man with pulmonary nodules whose histological analysis initially suggested tuberculosis. The Mycobacterium tuberculosis (MT) culture was negative and a questionnaire revealed a professional activity of brushing and polishing surgical instruments without any protection for 7 years.  A mineralogical analysis by optical and electron microscopy was performed on both a healthy lung tissue biopsy and a lung nodule in a paraffin block. Electron microscopy analysis revealed the presence of metal particles (iron oxide, titanium oxide, aluminum oxide and steel) in both samples. This study suggests that mineralogical analysis combined with a questionnaire on dust exposure could help redirect the diagnosis of a dust-related disease.

  16. Lymphomatoid granulomatosis of the brain: A case report

    Directory of Open Access Journals (Sweden)

    Edgardo Quinones

    2016-01-01

    Conclusions: This rare lesion in an adult, immunocompetent patient, debuting with motor seizures represents a challenge in terms of diagnosis and treatment. After surgical and medical treatment, the patient had a satisfactory recovery. Clinical features, imaging, differential diagnosis, and pathology are discussed.

  17. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

    Science.gov (United States)

    Horowitz, Netanel; Ben-Itzhak, Ofer; Braun-Moscovici, Yolanda

    2016-01-01

    In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature. PMID:27293945

  18. Eosinophilic Granulomatosis with Polyangiitis, formerly Churg-Strauss Syndrome (EGPA)

    Science.gov (United States)

    ... Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], ... phase of the disease is often marked by eosinophilia, the finding of an excessive number of eosinophils ...

  19. Liver granulomatosis: a case of Chlamydophila pneumoniae infection.

    Science.gov (United States)

    Yildiz, H; Wieërs, G; Yombi, J C; Marot, J C

    2015-02-01

    An 18-year-old man was referred to the Internal Medicine ward because of a 2-week history of intermittent high fever, weight loss and cough. Clinical examination revealed hepato-splenomegaly and multiple lymph nodes swelling while laboratory tests showed elevated C-reactive protein, gamma glutamyl transferase and lactate dehydrogenase. All serologic testes for auto-immune antibodies, viruses and bacteria were negative except for Chlamydophila pneumoniae. An 18-FDG PET computed tomography scanner showed hypermetabolism in the liver, spleen and lymph nodes. We therefore conducted a liver biopsy that demonstrated non-necrotizing granulomas. We conclude to a C. pneumoniae infection associated with a granulomatous hepatitis. After treatment with Doxyciclin the patient had no more fever, hepatosplenomegaly resolved and blood testes normalized. This case report is to our knowledge the first report of a granulomatous hepatitis associated with C. pneumoniae respiratory infection.

  20. Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

    Directory of Open Access Journals (Sweden)

    Mohammad E. Naffaa

    2016-01-01

    Full Text Available In a patient with systemic multiorgan disease with overlapping features, the differential diagnosis included infectious diseases, malignancies, and systemic autoimmune or inflammatory diseases. We present an unusual case of a young male with B cell lymphoma who presented with symptoms mimicking systemic vasculitis and review the existing literature.

  1. 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.

  2. Meningitis granulomatosa, glomerulonefritis rápidamente progresiva y vasculitis

    Directory of Open Access Journals (Sweden)

    Ana Ludueña

    2011-08-01

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

  3. [Safety and efficacy of biologics directed against TNF-alpha and CD20 in the therapy of vasculitis and systemic lupus erythematosus].

    Science.gov (United States)

    Walker, Ulrich A; Hasler, Paul

    2008-05-01

    While the inhibition of TNF-alpha has been shown to improve vasculitis in vitro and in animal models, the clinical evidence for the efficacy of TNF-alpha blockade in most forms of vasculitis is mainly based on case reports and case series. Randomised controlled studies have so far not shown superiority of anti-TNF-alpha agents for Wegener's granulomatosis and giant cell arteritis. Moreover, in the context of Wegener's granulomatosis, a higher frequency and severity of infections are to be expected. In refractory cases of Behçet's disease therapy of uveitis and other organ manifestations is promising. Rituximab has achieved good effects in case reports of vasculitis. Results from controlled trials are not available. Observational studies indicate that in refractory systemic lupus erythematosus, and possibly also in several instances of small vessel vasculitis, rituximab can achieve good responses. The increased frequency of severe infections under TNF-alpha blockade requires a stringent benefit and risk assessment in addition to a multidisciplinary analysis of follow-up parameters. A detailed information of the patient regarding symptoms and signs of a possible infection are a prerequisite. Due to the complexity of the field and the danger of morbidity and mortality as a consequence of vasculitis or systemic lupus erythematosus on the one hand, and of the therapy on the other, biologics should only be used to treat these disorders in institutions fully equipped and staffed for this purpose.

  4. 心脏韦格纳肉芽肿1例临床病理观察

    Institute of Scientific and Technical Information of China (English)

    姚庆端; 张金升; 于海易; 王敏; 曲美婷

    2004-01-01

    To investigates the clinical sings and the pathology morphologic changes of the heart tissue of a case with the cardiac Wegener' s granulomatosis. Methods The clinical with was examined ECG and laboratory. The pathology samples were sectioned routine paraffin and stained with HE、PAS、AB-PAS and cellulose staining and observed with light microscope. Results The ECG was manifested acute myocardial infarction in the left lateral wall and the blood examination were of the heart, and the blood examination were manifested lift pedigree ferment of myocardium. The pathology sections were manifested necrosis large area perforation wall of the myocardial and became granuloma diffusible. Endocarditis, necrotic vesselitis and thrombus could be found and that the coronary is not embolism. Similar lesions could be found and alike to the heart in the left lung parenchyma and the peripheral zone of trachea and blood vessel. Histochemical examination with PAS and AB-PAS showed cryptococcus negative in the heart and lungs. Cellulose staining was positive reaction in the vascular wall. Antineutrophil cytoplasmic antibody (ANCA) was negative in the blood from the heart. Conclusion Cardiac Wegener's granulomatosis is rare. Clinical sings the ever so similar to acute myocardial infarction and that is not all but differentiation and prognosis and unfavorable of the ever cuch.

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

    Science.gov (United States)

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

    2011-01-01

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

  6. Risk factors of atherosclerosis and clinical and morphological comparisons in systemic vasculitides

    Directory of Open Access Journals (Sweden)

    Leonid Aleksandrovich Strizhakov

    2012-01-01

    Full Text Available Objective: to study the incidence rates of angina, myocardial infarction (MI, stroke, and the frequency of endovascular interventions in patients with systemic vasculitides, and the incidence rate of atherosclerosis according to autopsy data. Subjects and methods. Three hundred and twenty-one patients with systemic vasculitides: Wegener's granulomatosis (n = 138, Takayasu's arteritis (n = 79, polyarteritis nodosum (n = 55, and Churg-Strauss syndrome (n = 49 were examined; 55 autopsies were analyzed in patients with the above diseases. Results. Fifty-one (15.6% of the 321 patients were diagnosed as having cardiovascular diseases (CVD: angina pectoris (7.1% and MI (3.1% and endovascular interventions (0.9%. The risk of cardiovascular events was found to be associated with traditional risk factors, such as male gender and age. Arterial hypertension, hypercholesterolaemia, and increased serum creatinine were more frequently detected in the CVD group that showed no significant differences from the non-CVD group. According to autopsy results, atherosclerosis was identified in the patients with Wegener's granulomatosis (52%, Takayasu's arteritis (50%, polyarteritis nodosum (52.6%, and Churg-Strauss syndrome (57.1%. Conclusion. CVD and atherosclerosis are common in systemic vasculitides, which requires the traditional risk factors of atherosclerosis to be actively corrected.

  7. Categorisering van wegen en gewenste rijsnelheden : een discussienota ten behoeve van de Werkgroep "Rijsnelheid" van Rijkswaterstaat.

    NARCIS (Netherlands)

    Janssen, S.T.M.C. Roszbach, R. Noordzij, P.C. & Wesemann, P.

    1992-01-01

    The Ministry of Transport and Public Works Working Group `Driving speed' was commissioned to perform an exploration into a desirable speed limit per road category. For this purpose propositions are made for a classification system with accompanying speed limits for the road network in The Netherland

  8. Samenhang tussen ongevallenrisico, snelheid en wegkenmerken op 80km/uur-wegen : een steekproefstudie.

    NARCIS (Netherlands)

    Aarts, L.T. & Schagen, I.NL.G. van

    2007-01-01

    The relation between crash rate, speed and road features on 80 km/h roads; a cross-sectional study. This exploratory study investigates the relation between crash rate, speed, and several features of 80 km/h roads. We carried out this study with driving speed and crash data available to us from anot

  9. De veiligheid van bermen en wegen : een beschouwing van de stand van zaken.

    NARCIS (Netherlands)

    Heijer, T.

    1992-01-01

    By order of the Dienst Verkeerskunde of the Ministry of Transport and Public Works, the current situation around the safety of road side safety constructions in the Netherlands and the possible consequences of a number of future developments were analyzed by SWOV Institute for Road Safety Research.

  10. The three lost millennia of the last deglaciation (Alfred Wegener Medal Lecture)

    Science.gov (United States)

    Bard, Edouard

    2013-04-01

    Looking back more than thirty years, climate history over the last period of deglaciation was seen to portray a smooth transition between the last glacial maximum (LGM) centered around 18,000 years ago (based on radiocarbon), and the beginning of the Holocene at about 10,000 years before present. At that time, the renowned CLIMAP group used the stratigraphy available to reconstruct the glacial world by averaging paleothermometric data over a wide time window, ranging between at least 14,000 and 24,000 yr BP, over which period climate was assumed to be rather stable. Even if northern European pollen records showed several phases of vegetation shift, the exact duration and spatial coverage of these shifts was unknown and their climatic significance was not well-enough understood to be separated from other biological effects, such as plant migration following ice-sheet demise. Significant progress came from mass spectrometry developments applied to isotope geochronology in the mid- and late- 1980s. This allowed the precise analysis of radiocarbon on small samples such as foraminifera in marine sediments and enabled the measurement of U-Th ages for accurate dating of corals and speleothems. These technological improvements permitted meaningful comparisons between proxy records from the various archives originating from all latitudes and longitudes. Today, it is clear that the old LGM time window corresponds to a period of more than ten millennia during which there was significant climate variability, including a prominent cooling event at the beginning of the deglaciation. This cooling event is known as the 'Oldest Dryas' by palynologists, as 'Heinrich Event #1' (H1) by paleoceanographers, and has even been dubbed the 'Mystery Interval' by prominent authors as they puzzled while attempting to synthesize and interpret its records. The H1 drastic cooling, attributed to a pulse-like injection of ice and meltwater into the North Atlantic, was first evidenced in 1987 in sediments from the Iberian Margin. Three years later, significant improvements of the radiocarbon calibration demonstrated that about three millennia were missing from the deglaciation record. Accordingly, the LGM mean age was pushed from 18,000 to 21,000 yr BP, the midpoint of H1 was shifted from 13,500 to 16,000 yr BP, and the beginning of the Holocene was repositioned at about 11,500 yr BP. This new climate chronology was subsequently confirmed by counting 'cryovarves' within the GRIP and GISP2 Greenland ice cores. These studies have since been complemented by many other records from polar ice, marine and lacustrine sediments and cave speleothems. In addition to extending the chronology by three additional millennia, improvement also arose from the quality of the new geological archives. These archives have allowed studies at much higher resolution than was previously achieved in the framework of CLIMAP, which included many records based on deep-sea cores characterized by low sedimentation rate, and thus very susceptible to smoothing processes such as bioturbation. In addition, analytical geochemistry has only recently provided techniques adapted to the production of high-resolution time series of various proxies based on elemental ratios, on organic compounds or on stable and radiogenic isotopes. More than a dozen years after the H1 discovery, the same Iberian Margin sediments were used to show that H1 comprised at least two phases, H1a and H1b, based on ice rafted debris (IRD) and other proxies. It is now recognized that the entire H1 event (H1 sensu lato) is a three millennia-long period (ca. 17,500 to 14,500 yr BP). To illustrate the progress in this research field, I will review the key records that can be used to document the complex nature of this episode. The H1 (s.l.) included several phases of intense cooling, of precipitation changes - notably at low latitudes and in the Asian monsoon area, of retreat and decay of glacial ice-sheets - as evidenced in sediments collected in river mouths, and of sea-level rise as recorded in corals from Tahiti and Barb

  11. Improving air quality (policy) around roads; Naar een beter luchtkwaliteitsbeleid rond wegen

    Energy Technology Data Exchange (ETDEWEB)

    Van Dam, C.E.; Kruyt, H.A.; Dorel, F.G. [Directie Water en Milieu, Provincie Zuid-Holland, The Hague (Netherlands)

    1997-12-01

    Due to the ever increasing traffic in the Netherlands air quality around roads will remain poor, in spite of measures taken to reduce the emissions of both industry and traffic (e.g catalysts). To improve air quality policy, and thereby air quality, it is required (1) to integrate air quality policy with other environmental policy aspects at an early stage; (2) that the legal air quality standards are accounted for during the process of physical planning by municipalities and provinces; (3) that all three organisations who are involved in road-maintenance (state, province, municipality) also report about air pollution levels around those roads; (4) to develop a new mathematical model with which local air pollution can be reliably estimated for future situations (e.g. in 2010); (5) to introduce an air quality standard for fine dust. Although the emphasis in air quality policy should be on reducing emissions, it seems that in addition a remediation policy should be developed and implemented. This policy should aim at reducing the amount of people exposed to too much air pollution as much as possible. 8 refs.

  12. Natuurlijk sturen in Limburg : een kijkgedrag- en snelheidsonderzoek en een verkeerskundige analyse van twee aangepaste wegen.

    NARCIS (Netherlands)

    Vlakveld, W.P. Boele, M.J. Aarts, L.T. & Schermers, G.

    2013-01-01

    Natural traffic calming in the Dutch Province of Limburg : pilot study of two adapted roads. The continuous search for infrastructural measures to further improve road safety motivated and stimulated a number of regions to use more ‘natural’ measures and territorial characteristics. These natural

  13. Een categorie-indeling van wegen benaderd vanuit het aspect verkeersveiligheid.

    NARCIS (Netherlands)

    Janssen, S.T.M.C.

    1977-01-01

    Foreseeing the consequences when manoeuvring is easier when traffic situations are not too complex. This can be achieved by categorization of roads. Within one road category roads must be consistent, continuous, uniform and recognizable in their characteristics. The traffic area can be divided in

  14. De gemiddelde reissnelheid op auto (snel) wegen : kwantificering van vertragingen door diverse oorzaken.

    NARCIS (Netherlands)

    Burgt, G.J. van der Hammendorp, H.J. & Kampen, L.T.B. van

    1972-01-01

    This article discusses the results of a study of car average travelling speeds on highways in the Netherlands. Test runs were made with desired speeds of 80, 110, and 140 km/h after which time losses caused by road and traffic characteristics were analyzed. Recommendations are given for the design o

  15. Snelheid en verkeersonveiligheid op 80 km/uur-wegen : een literatuurstudie.

    NARCIS (Netherlands)

    Oei, H.-l.

    1990-01-01

    The aim of this literature review was to make an inventory of theoretical and empirical knowledge with regard to speed in its relationship to the characteristics of the highway, the traffic and the driver. Results show that speed control has great potential possibilities to improve traffic safety.

  16. Die DDC auf neuen Wegen - verbale Sucheinstiege für klassifikatorisch erschlossene Titel

    Directory of Open Access Journals (Sweden)

    Christiane Maibach

    2014-12-01

    Full Text Available Die Dewey-Dezimalklassifikation (DDC ist ein mächtiges Instrument der klassifikatorischen Inhaltserschließung. Immer mehr Bibliotheken im In- und Ausland erkennen den Nutzen der international am weitesten verbreiteten Universalklassifikation. Die Deutsche Nationalbibliothek (DNB setzt die DDC seit 2006 für die inhaltliche Erschließung der Bibliografiereihen A, B und H ein. Seitdem wurden rund 800.000 Publikationen mit DDC-Notationen versehen. Der Nutzen von Klassifikationen ist unter Bibliothekar/innen unumstritten. Die Bibliotheksnutzer/innen hingegen wissen oft nicht, dass der Katalog ihrer Bibliothek auch eine systematische Recherche ermöglicht. Ihre Recherchestrategie ist meist verbal. Durch Internetsuchmaschinen wird diese Strategie noch bestärkt. Daher liegt es nahe, auch für die klassifikatorisch erschlossenen Titel einen verbalen Sucheinstieg zu schaffen. Die DDC enthält nicht nur die in vielen Klassifikationssystemen üblichen Registereinträge und Klassenbenennungen, sondern auch die im Rahmen des Projekts CrissCross in großem Umfang erstellten Verknüpfungen zu Schlagwörtern der Gemeinsamen Normdatei (GND, die für diesen Zweck ausgewertet werden können. The Dewey Decimal Classification (DDC is a powerful indexing tool. A rising number of libraries worldwide recognize the benefit of this universal classification. Since 2006 the German National Library (DNB has used the DDC to index titles belonging to series A, B and H of the German National Bibliography. In 2012 100,000 publications were indexed with DDC notations. The benefits of classifications for indexing is widely accepted among librarians. However, library users take a different point of view. Mostly, they are not even aware that their library's catalogue offers a systematic search. Most library users prefer verbal search strategies. This is enforced by internet search engines, which have changed the search habits of library users. Therefore, the obvious solution is to create a verbal access to classified titles. The DDC does not only contain a relative index with verbal elements and class names, but also links to subject headings taken from the German Integrated Authority File (GND. These were established as a part of the CrissCross project. All of these verbal elements can be used to make the DDC accessible by words.

  17. Invasive pulmonary mycosis due to Chaetomium globosum with false-positive galactomannan test: a case report and literature review.

    Science.gov (United States)

    Capoor, Malini R; Agarwal, Poojan; Goel, Manoj; Jain, Sarika; Shivaprakash, Mandya Rudramurthy; Honnavar, Prasanna; Gupta, Sunita; Chakrabarti, Arunaloke

    2016-03-01

    In this case, the authors report Chaetomium globosum as a cause of invasive pulmonary infection in a patient with Wegener's granulomatosis. Fungal hyphae (KOH and Calcofluor) were seen on direct microscopy of lung biopsy sample and bronchoalveolar lavage (BAL) sample. C. globosum isolated on culture clinched the diagnosis of invasive pulmonary infection by Chaetomium spp. A positive galactomannan of serum and BAL was repeatedly seen and was utilised for follow-up and as prognostic marker in patient management. The patient was successfully treated with liposomal amphotericin B followed by voriconazole. All the Chaetomium infections reported till date since 1980 are reviewed. Chaetomium spp. with its unique ecology has a hidden clinical potential to cause invasive mould infections.

  18. The inspiratory "squawk" in extrinsic allergic alveolitis and other pulmonary fibroses.

    Science.gov (United States)

    Earis, J E; Marsh, K; Pearson, M G; Ogilvie, C M

    1982-01-01

    An inspiratory musical sound ("squawk") was recorded in 14 patients with diffuse pulmonary fibrosis. These were divided into two groups: nine patients suffering from extrinsic allergic alveolitis (seven with bird fancier's lung and two with farmer's lung) and five patients with pulmonary fibrosis due to other causes, including rheumatoid disease, Wegener's granulomatosis, systemic sclerosis, and sarcoidosis. Clinical studies and phonopneumographic analysis of 10 consecutive squawks in each patient showed that the sound in the group with extrinsic allergic alveolitis was of shorter duration, occurred later in inspiration, and tended to be of higher frequency than the sound heard in the other group. Inspiratory crackles were present in all patients and in eight a single loud crackle preceded the squawk. We suggest that squawks, like crackles, result from the opening of airways and that the differences between the squawks in the two groups may reflect the size of the affected airways. PMID:7170682

  19. 以呕吐为首发症状的老年ANCA相关性血管炎两例分析

    Institute of Scientific and Technical Information of China (English)

    孙德珍

    2009-01-01

    @@ ANCA相关性血管炎(ANCA associated vasculitis) 主要包括韦格纳肉芽肿(wegener's granulomatosis,WG)和显微镜下多动脉炎(mycroscopic polyangilitis,MPA).因其与抗中性粒细胞胞浆抗体(ANCA)密切相关,故又称ANCA相关性系统性血管炎[1].本病多见于老年病人,高峰发病年龄为55~70岁[2].MPA是一种主要累及小血管的系统性坏死性血管炎,常表现为坏死性肾小球肾炎和肺毛细血管炎.临床上以血尿和咯血为主要症状.

  20. Imaging appearance of granulomatous lesions of head and neck

    Energy Technology Data Exchange (ETDEWEB)

    Abdel Razek, Ahmed Abdel Khalek, E-mail: arazek@mans.eun.e [Diagnostic Radiology Department, Mansoura Faculty of Medicine, 62 ElNokrsi Street-Meet Hadr, Mansoura (Egypt); Castillo, Mauricio [Department of Radiology, University of North Carolina, Chapel Hill, NC (United States)

    2010-10-15

    We aim to review the imaging appearance of granulomatous lesions of the head and neck. Granulomatous lesions are seen in different regions of the head and neck and a difficult diagnostic challenge for the radiologist. Infective granulomas may be due to bacterial or fungal agents. Non-infective granulomas are Wegener's granulomatosis, sarcoidosis, amyloidosis, chemical granuloma and reparative giant cell granuloma. Familiarity with the clinical presentation and imaging features of these lesions can suggest diagnosis in some cases. CT and MR imaging demonstrate the exact location, extension and effect on surrounding structures. A thorough knowledge of age, gender, common location, clinical features and imaging appearance of granulomatous lesions are important for diagnosis.

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

    OpenAIRE

    María Virginia Paolini; Juan Pablo Ruffino; Diego S. Fernández Romero

    2013-01-01

    Las vasculitis asociadas a anticuerpos anti-citoplasma de neutrófilos (ANCA) comprenden a un grupo de enfermedades caracterizadas por la inflamación de la pared de pequeños vasos. Analizamos las características epidemiológicas y clínicas en una serie de 47 pacientes: 23 (49%) granulomatosis de Wegener (GW), 15 (32%) poliangeítis microscópica (PAM) y nueve (19%) vasculitis limitada al riñón (VLR). La edad media al inicio de los síntomas fue de 50.7 ± 14.9 años. La manifestación clínica más fre...

  2. [Skin changes in rheumatic diseases].

    Science.gov (United States)

    Dobrić, Ivan

    2005-01-01

    The Intruduction includes those eflorescences that might be useful for diagnostics in rheumatology. Further in the text we have described four groups of rheumatic disorders. The first group: rheumatic diseases (lupus erythematosus, dermatomyositis, systemic scleroderma, the mixed connective tissue disease, allergic vasculitis, polyarteritis) which are the most common from the dermatological point of view. The second group: rheumatic diseases (Wegener's granulomatosis, rheumatoid arthritis, Sjögren, Reiter and Behçet syndrome and Kawasaki's disease) which are rarely of interest to our dermatologists. In this group there is also psoriatic arthritis, which is not rare in dermatology but its diagnostics and treatment belong to rheumatologists' field of expertise. The third group: infections (rheumatic fever, diseminated gonococcal infection, subacute bacterial endocarditis, Lyme disesease). The fourth group: metabolic disorders (gout). The diseases of the first group are described completely. In the second, third and fourth group of the diseases we have included only skin changes.

  3. Fatal inflammatory hypophysitis.

    Science.gov (United States)

    McIntyre, Elizabeth A; Perros, Petros

    2007-01-01

    A young female patient presented as an acute medical emergency with hypoglycaemia. Investigations revealed panhypopituitarism and an inflammatory pituitary mass. An antibody screen was negative for anti-neutrophil cytoplasmic antibodies with cytoplasmic distribution (cANCA). Pituitary histology showed lymphocytic infiltration and a few Langerhan's cells. The pituitary mass rapidly expanded to involve the optic nerves and led to bilateral blindness. Later, the patient developed diarrhoea, a vasculitis rash, scleritis, and proteinuria. In subsequent investigations cANCA became positive. The patient responded to steroids and cyclophosphamide treatment and remained in partial remission for six months before dying of severe sepsis. This is the first description of Wegener's granulomatosis presenting with acute anterior pituitary failure in the absence of other organ involvement and negative serology.

  4. Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody.

    Science.gov (United States)

    Yokoseki, Akiko; Saji, Etsuji; Arakawa, Musashi; Kosaka, Takayuki; Hokari, Mariko; Toyoshima, Yasuko; Okamoto, Kouichirou; Takeda, Shigeki; Sanpei, Kazuhiro; Kikuchi, Hirotoshi; Hirohata, Shunsei; Akazawa, Kouhei; Kakita, Akiyoshi; Takahashi, Hitoshi; Nishizawa, Masatoyo; Kawachi, Izumi

    2014-02-01

    The aim of this study was to elucidate the characteristics, pathogenesis and treatment strategy of hypertrophic pachymeningitis that is associated with myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). We retrospectively investigated clinical, radiological, immunological and pathological profiles of 36 patients with immune-mediated or idiopathic hypertrophic pachymeningitis, including 17 patients with myeloperoxidase-ANCA, four patients with proteinase 3-ANCA, six patients with other immune-mediated disorders, and nine patients with 'idiopathic' variety. Myeloperoxidase-ANCA-positive hypertrophic pachymeningitis was characterized by: (i) an elderly female predominance; (ii) 82% of patients diagnosed with granulomatosis with polyangiitis (previously known as Wegener's granulomatosis) according to Watts' algorithm; (iii) a high frequency of patients with lesions limited to the dura mater and upper airways, developing headaches, chronic sinusitis, otitis media or mastoiditis; (iv) a low frequency of patients with the 'classical or generalized form' of granulomatosis with polyangiitis involving the entire upper and lower airways and kidney, or progressing to generalized disease, in contrast to proteinase 3-ANCA-positive hypertrophic pachymeningitis; (v) less severe neurological damage according to the modified Rankin Scale and low disease activity according to the Birmingham Vasculitis Activity Score compared with proteinase 3-ANCA-positive hypertrophic pachymeningitis; (vi) increased levels of CXCL10, CXCL8 and interleukin 6 in cerebrospinal fluids, and increased numbers of T cells, neutrophils, eosinophils, plasma cells and monocytes/macrophages in autopsied or biopsied dura mater with pachymeningitis, suggesting TH1-predominant granulomatous lesions in hypertrophic pachymeningitis, as previously reported in pulmonary or renal lesions of granulomatosis with polyangiitis; and (vii) greater efficacy of combination therapy with prednisolone and

  5. Role of radiotherapy in the treatment of granuloma gangraenescens (lethal midline granuloma)

    Energy Technology Data Exchange (ETDEWEB)

    Sack, H.; Horch, H.H.; Schaefer, H.E.; Wustrow, F.

    1984-02-01

    The granuloma gangraenescens in the oral, maxillary and facial region is a rare disease. It is a destroying process in the region of palate, nose, paranasal sinuses, cheeks and orbit with characteristic signs of granulomatosis, infection and malignancy. The disease shows often a lethal development with cachexia or sepsis. Besides local inflammations and tumors, the granulomatosis of Wegener, and the so-called necrotizing sialometaplasia are above all to be excluded by differential diagnosis. Five cases are presented in order to describe the diagnostic and therapeutic problems. In literature, the greatest efficacy is attributed to radiotherapy, however, a detailed definition of the most efficient irradiation conditions cannot be given yet because of the small number of cases. Good long-term results or recoveries can be achieved in 75 to 80% of cases by a relatively high radiation dose of 40 to 50 Gy administered within four to five weeks. During the observation time of two to six years, no one of the five patients treated here only by megavoltage therapy showed a recurrence.

  6. 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.

  7. Immunoadsorption in Autoimmune Diseases Affecting the Kidney.

    Science.gov (United States)

    Stummvoll, Georg; Aringer, Martin; Handisurya, Ammon; Derfler, Kurt

    2017-09-01

    Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Although past data have shown efficacy and biocompatibility of immunoadsorption in (renal) SLE, it is still an experimental and expensive procedure, and evidence from randomized controlled trials is needed. Nevertheless, immunoadsorption is being used as a rescue therapy in life-threatening situations of SLE patients because of its fast mode of action and its acceptable safety profile. In granulomatosis with polyangiitis (GPA) (or Wegener's granulomatosis), microscopic polyangiitis (MPA), and anti-glomerular basement membrane disease, the current standard is plasma exchange. Immunoadsorption, which probably would reduce the autoantibody burden more effectively, might be an even better more effective option, but sufficient evidence is lacking. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Systemic Lupus Erythematosus and hearing disorders: Literature review and meta-analysis of clinical and temporal bone findings.

    Science.gov (United States)

    Di Stadio, Arianna; Ralli, Massimo

    2017-01-01

    Objective This literature review and meta-analysis was performed to evaluate the correlations among hearing and vestibular clinical symptoms, temporal bone findings, and pathological mechanisms in patients with systemic lupus erythematosus (SLE). Study design Relevant papers in the literature were retrospectively reviewed. Clinical hearing aspects in patients with SLE and relevant temporal bone studies in the same field were analyzed. Methods PubMed and Google Scholar searches were performed using the following keywords: "auto-immune disease," "systemic lupus erythematosus (SLE)," "hearing loss," "temporal bone study," "vertigo," "dizziness," "tinnitus," "ear symptoms," "treatment," "diagnosis," "symptoms," "etiopathogenesis," "Wegener granulomatosis," "Sjogren," "polyarteritis nodosa," "Cogan syndrome," and "granulomatosis." Also included were reviews in which the following terms were present: "SLE," "temporal bone," and "hearing symptoms." Review and conclusion This literature review and meta-analysis focused on the pathological mechanisms through which SLE can damage inner ear structures and determinate hearing and vestibular symptoms. The main mechanisms involved in inner ear damage include the autoimmune response, deposition of immune complexes in the vessels and, to a lesser extent, cytotoxic damage.

  9. Childhood vasculitis hospitalizations in Spain, 1997-2011.

    Science.gov (United States)

    Villaverde-Hueso, A; Alonso, V; Morales-Piga, A; Hens-Pérez, M; Abaitua, I; Posada-de-la-Paz, M

    2014-05-01

    The aim of this study is to describe the childhood vasculitis hospital burden in Spain (1997-2011), considering type of disease, hospitalization rates and time trends. Data were obtained from the National Discharges Basic Minimum Data Set (National Patient Data Base). Inpatient events of children younger than 15 years of age were analyzed. Principal diagnosis of vasculitis were selected according Ninth Revision of the International Classification of Diseases: Takayasu arteritis, Polyarteritis nodosa, Kawasaki disease, Wegener`s granulomatosis, Churg-Strauss syndrome, and Henoch-Schönlein purpura. A total of 14518 children hospitalizations related to vasculitis were identified in Spain from 1997 to 2011. The average hospitalization rate for children was 13.33±1.71 per 100,000. Henoch-Schönlein purpura and Kawasaki disease were the most common type of vasculitis, hospitalization rates were 11.00 and 3.97 per 100,000 children, respectively. Other vasculitis hospitalizations are much rare in childhood. Average length of stay was 6.04 days and estimated cost per inpatient hospital care was 2,847€. Hospital case fatality rate was 0.05% for overall vasculitis. In conclusion, epidemiological data of childhood vasculitis are useful both to health decision-making and to identify research priorities.

  10. 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.

  11. A pragmatic approach to vasculitis in the gastrointestinal tract.

    Science.gov (United States)

    Chetty, Runjan; Serra, Stefano

    2017-06-01

    Although vasculitis involving the gastrointestinal tract (GIT) is an uncommon occurrence, occasionally vasculitis can present as haemorrhagic infarction or ischaemia for which a length of bowel is removed. Invariably, the appropriate clinical history is not forthcoming, or vasculitis is not clinically suspected. The purpose of this overview is to provide the practising gastrointestinal (GI) pathologist with a framework to recognise and diagnose vasculitides within the GIT. The classification may be approached by aetiological agent or size of vessel involved; an international consensus group now favours the latter approach. The symptoms that systemic and/or localised vasculitis may cause in the GIT are protean and non-specific. As a result, pathologists examining resection specimens for unexplained haemorrhagic infarction or ischaemia should be aware that vasculitis may be a potential cause. Several well-known systemic vasculitides such as polyarteritis nodosa, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome and granulomatosis with polyangiitis or Wegener's granulomatosis can occur in the GIT. The latter three constitute the antineutrophil cytoplasmic antibody-positive vasculitides. In addition, the so-called solitary organ vasculitis (SOV) can occur in the GIT as the harbinger of later onset systemic vasculitis, and be the cause of the GIT symptoms. In addition, SOV can occur incidentally and coexist with GIT disease such as gallstones or polyps, and there may be no manifestations of systemic vasculitis for years, or not at all. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  12. Ultrastructural characteristics of type A epithelioid cells during BCG-granulomatosis and treatment with lysosomotropic isoniazid.

    Science.gov (United States)

    Shkurupii, V A; Kozyaev, M A; Nadeev, A P

    2006-04-01

    We studied BCG-granulomas, their cellular composition, and ultrastructure of type A epithelioid cells in the liver of male BALB/c mice with spontaneous granulomatous inflammation. The animals received free isoniazid or isoniazid conjugated with lysosomotropic intracellularly prolonged matrix (dialdehyde dextran, molecular weight 65-75 kDa). Lysosomotropic isoniazid was accumulated in the vacuolar apparatus of epithelioid cells and produced a stimulatory effect on plastic processes in these cells.

  13. Acute Myocarditis in a Patient with Newly Diagnosed Granulomatosis with Polyangiitis

    DEFF Research Database (Denmark)

    Munch, Anne; Sundbøll, Jens; Høyer, Søren;

    2015-01-01

    (MRI) showed findings consistent with myocarditis but the etiology of the apical hypokinesis could not be determined with certainty and may well have been due to a myocardial infarction, a notion supported by a coronary angiogram displaying slow flow in the territory of the left anterior descending...... artery. Finally, an endomyocardial biopsy confirmed the diagnosis of myocarditis. The cardiac symptoms subsided upon treatment with high-dose prednisolone and rituximab....

  14. A young lady presented with limited pulmonary Wegener′s granulomatosis

    Directory of Open Access Journals (Sweden)

    Dey Atin

    2008-01-01

    Full Text Available A 19 year old female college student presented with fever, dry cough, chest pain, blood tinged sputum with subsequent development of polyarthralgia with radiological evidence of bilateral multiple unevenly distributed pulmonary nodular opacities with cavitation. There was no other systemic involvement and the patient was cytoplasmic antineutrophil antibody (c-ANCA positive with more than four times the normal upper limit of anti PR3 antibody. Excellent response to oral steroid with antimicrobial agent "trimethoprim - sulphamethoxazole" was noted.

  15. Cutaneous granulomatosis and combined immunodeficiency revealing Ataxia-Telangiectasia: a case report

    OpenAIRE

    Antoccia Antonio; Ferrari Francesca; Angelino Giulia; Scarselli Alessia; Folgori Laura; Chessa Luciana; Finocchi Andrea

    2010-01-01

    Abstract Ataxia-telangiectasia (A-T) is a complex multisystem disorder characterized by progressive neurological impairment, variable immunodeficiency and oculo-cutaneous telangiectasia. A-T is a member of chromosomal breakage syndromes and it is caused by a mutation in the ataxia-telangiectasia mutated (ATM) gene. Because of a wide clinical heterogeneity, A-T is often difficult to diagnose in children. We report an unusual case of a 3-year-old boy affected by A-T who presented exclusively wi...

  16. De relatie tussen snelheidslimiet en verkeersveiligheid : onwikkeling van de verkeersonveiligheid op 50- en 80km/uur-wegen vergeleken met die op 30- en 60km/uur-wegen.

    NARCIS (Netherlands)

    Braimaister, L. Bos, N.M. Kars, V. & Stipdonk, H.L.

    2013-01-01

    The relation between speed and road safety : the development of road safety on 50 and 80 km/h roads compared with the development on 30 and 60 km/h roads. Since the implementation of the Start-up Programme Sustainable Safety from 1998 onward, the speed limit on a considerable length of urban access

  17. De relatie tussen snelheidslimiet en verkeersveiligheid : onwikkeling van de verkeersonveiligheid op 50- en 80km/uur-wegen vergeleken met die op 30- en 60km/uur-wegen.

    NARCIS (Netherlands)

    Braimaister, L. Bos, N.M. Kars, V. & Stipdonk, H.L.

    2013-01-01

    The relation between speed and road safety : the development of road safety on 50 and 80 km/h roads compared with the development on 30 and 60 km/h roads. Since the implementation of the Start-up Programme Sustainable Safety from 1998 onward, the speed limit on a considerable length of urban access

  18. Rijsnelheden op 80 km/uur-wegen in Nederland II : verslag van snelheidsmetingen in twaalf geografisch gespreide gebieden in Nederland.

    NARCIS (Netherlands)

    Oei, H.-l. & Pol, W.H.M. van de

    1991-01-01

    An account is given of radar measurements of driving speeds on 80 km/h roads carried out in twelve areas in the Netherlands, in October 1990. The most important results of these measurements are as follows: : (1) The average speed on 80 km/h roads in the twelve regions appeared to be 77 km/h; (2) Th

  19. From Engineering Hydrology to Earth System Science: Milestones in the Transformation of Hydrologic Science (Alfred Wegener Medal Lecture)

    Science.gov (United States)

    Sivapalan, Murugesu

    2017-04-01

    Hydrologic science has undergone almost transformative changes over the past 50 years. Huge strides have been made in the transition from early empirical approaches to rigorous approaches based on the fluid mechanics of water movement on and below the land surface. However, further progress has been hampered by problems posed by the presence of heterogeneity, especially subsurface heterogeneity, at all scales. The inability to measure or map subsurface heterogeneity everywhere prevented further development of balance equations and associated closure relations at the scales of interest, and has led to the virtual impasse we are presently in, in terms of development of physically based models needed for hydrologic predictions. An alternative to the mapping of subsurface heterogeneity everywhere is a new earth system science view, which sees the heterogeneity as the end result of co-evolutionary hydrological, geomorphological, ecological and pedological processes, each operating at a different rate, which have helped to shape the landscapes that we see in nature, including the heterogeneity below that we do not see. The expectation is that instead of specifying exact details of the heterogeneity in our models, we can replace it, without loss of information, with the ecosystem function they perform. Guided by this new earth system science perspective, development of hydrologic science is now guided by altogether new questions and new approaches to address them, compared to the purely physical, fluid mechanics based approaches that we inherited from the past. In the emergent Anthropocene, the co-evolutionary view is expanded further to involve interactions and feedbacks with human-social processes as well. In this lecture, I will present key milestones in the transformation of hydrologic science from Engineering Hydrology to Earth System Science, and what this means for hydrologic observations, theory development and predictions.

  20. The Challenges of Developing a Framework for Global Water Cycle Monitoring and Prediction (Alfred Wegener Medal Lecture)

    Science.gov (United States)

    Wood, Eric F.

    2014-05-01

    The Global Earth Observation System of Systems (GEOSS) Water Strategy ("From Observations to Decisions") recognizes that "water is essential for ensuring food and energy security, for facilitating poverty reduction and health security, and for the maintenance of ecosystems and biodiversity", and that water cycle data and observations are critical for improved water management and water security - especially in less developed regions. The GEOSS Water Strategy has articulated a number of goals for improved water management, including flood and drought preparedness, that include: (i) facilitating the use of Earth Observations for water cycle observations; (ii) facilitating the acquisition, processing, and distribution of data products needed for effective management; (iii) providing expertise, information systems, and datasets to the global, regional, and national water communities. There are several challenges that must be met to advance our capability to provide near real-time water cycle monitoring, early warning of hydrological hazards (floods and droughts) and risk assessment under climate change, regionally and globally. Current approaches to monitoring and predicting hydrological hazards are limited in many parts of the world, and especially in developing countries where national capacity is limited and monitoring networks are inadequate. This presentation describes the developments at Princeton University towards a seamless monitoring and prediction framework at all time scales that allows for consistent assessment of water variability from historic to current conditions, and from seasonal and decadal predictions to climate change projections. At the center of the framework is an experimental, global water cycle monitoring and seasonal forecast system that has evolved out of regional and continental systems for the US and Africa. The system is based on land surface hydrological modeling that is driven by satellite remote sensing precipitation to predict current hydrological conditions, flood potential and the state of drought. Seasonal climate model forecasts are downscaled and bias-corrected to drive the land surface model to provide hydrological forecasts and drought products out 6-9 months. The system relies on historic reconstructions of water variability over the 20th century, which forms the background climatology to which current conditions can be assessed. Future changes in water availability and drought risk are quantified based on bias-corrected and downscaled climate model projections that are used to drive the land surface models. For regions with lack of on-the-ground data we are field-testing low-cost environmental sensors and along with new satellite products for terrestrial hydrology and vegetation, integrating these into the system for improved monitoring and prediction. At every step there are scientific challenges whose solutions are only partially being solved. In addition there are challenges in delivering such systems as "climate services", especially to societies with low technical capacity such as rural agriculturalists in sub-Saharan Africa, but whose needs for such information are great. We provide an overview of the system and some examples of real-world applications to flood and drought events, with a focus on Africa.

  1. De lichttechnische eigenschappen van lichtgekleurde wegoppervlakken : artikel Wegen 56 10: 803-824 t/m 803-325.

    NARCIS (Netherlands)

    Schreuder, D.A.

    1982-01-01

    Cement concrete road surfaces have, because of their light colour, a double luminance yield as compared with bituminous roads. Savings in electricity amount to at least 20% in illumination costs or 5-10% of total maintenance costs. Light colour improves visibility at night. Road markings in white

  2. DDR-Justiz und NS-Verbrechen Band 5 : Sammlung ostdeutscher Strafurteile wegen nationalsozialistischer Tötungsverbrechen

    NARCIS (Netherlands)

    Rüter, Christiaan F.

    2004-01-01

    This volume contains part of the trial cases finalized in the year 1951; the others are published in vol. IV. Again, a number of different criminal complexes are dealt with: - mass extermination crimes, - judicial crimes, - war crimes, - crimes committed in concentration camps and other detainment

  3. 5种ANCA相关性血管炎活动性评分量表的比较%Comparison of Five Different Disease Activity Scoring Scales for ANCA-associated Vasculitis

    Institute of Scientific and Technical Information of China (English)

    张立民; 吴庆军; 冯胜; 曾小峰

    2010-01-01

    目的 比较5种系统性血管炎活动性评分量表的可靠性及相关性,以指导临床应用.方法 以45例抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic autoantibody,ANCA)相关性血管炎(ANCA-associated vasculitis,AAV)患者为研究对象,其中包括韦格纳肉芽肿(wegener's granulomatosis,WG)患者30例和显微镜下多血管炎(microscopic polyangitis,MPA)患者15例.由2名评测者分别根据下述5种目前国际上常用的活动指数评价量表进行计分:伯明翰血管炎活动性评分(the Birmingham Vasculitis Activity Score,BVAS1994),韦格纳肉芽肿专用的BVAS(the BVAS for Wegener granulomatosis,BVAS/WG) ,BVAS2003,疾病累及范围指数(the Disease Extent Index,DEI),5因子评分(the Five Factor Score,FFS).结果 5种评分量表均有很高的重复信度(ICC 0.97~0.98)和评测者间信度(ICC 0.93~0.98).BVAS1994与BVAS2003(rs=0.961)、BVAS/WG(rs =0.872)、及DEI(rs=0.818),BVAS2003与BVAS/WG(rs=0.872)、DEI(rs=0.826),BVAS/WG与DEI(rs=0.776)间有较高的相关性,P值均小于0.001.FFS与其他评分量表之间无相关性.结论 目前AAV活动性评分量表是可靠、有效的.应用不同评分量表所得数据之间可以相互比较.

  4. Endoscope assisted coblation surgery for treating subglottic stenosis%内镜支撑喉镜下低温等离子射频治疗声门下狭窄

    Institute of Scientific and Technical Information of China (English)

    张庆丰; 张晶晶; 王慧

    2013-01-01

    Objective:To introduce a new surgical treatment for subglottic stenosis.Method:Six patients with subglottic stenosis were retrospectively studied.Four cases had history of intubation or tracheostomy.Two cases were because of B cell lymphoma and Wegener granulomatosis.All cases underwent endoscope assisted coblation surgery.Result:All of the patients were discharged from hospital with no tube in one week after surgery.In a follow-up of 3-21 months,stenosis in 2 cases of B cell lymphoma and Wegener granulomatosis recured 3 months after operation.These 2 patients were operated by coblation again and are still followed-up by now.Conclusion:The endoscope assisted coblation surgery is a new minimally invasive method fortreating subglottic stenosis.Further more,it can be reused to whom has a relapse.%目的:探讨内镜支撑喉镜下低温等离子射频治疗声门下狭窄的可行性及疗效.方法:回顾分析我院收治的6例声门下狭窄患者,继发于气管切开者1例,气管内插管后致狭窄者3例,声门下B细胞淋巴瘤1例,Wegener肉芽肿1例.6例均采用内镜支撑喉镜声门下狭窄低温等离子射频切除术.结果:术后1周内均成功拔管出院.随访3~21个月,声门下B细胞淋巴瘤及Wegener肉芽肿2例在术后3个月再次出现呼吸困难,并再次行低温等离子手术,目前拔管随访中.结论:内镜支撑喉镜下低温等离子射频治疗声门下狭窄是一种安全、微创的手术方式,对于再次狭窄的患者,可以再次手术.

  5. New Features of Disease after Diagnosis in Six Forms of Systemic Vasculitis

    Science.gov (United States)

    Grayson, Peter C.; Cuthbertson, David; Carette, Simon; Hoffman, Gary S.; Khalidi, Nader A.; Koening, Curry L.; Langford, Carol A.; Maksimowicz-McKinnon, Kathleen; Monach, Paul A.; Seo, Philip; Specks, Ulrich; Ytterberg, Steven R.; Merkel, Peter A.

    2015-01-01

    Objective To quantify the occurrence of features of vasculitis that initially present after diagnosis in 6 types of primary vasculitis. Methods Standardized collection of data on 95 disease manifestations in 6 vasculitides, including granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA), polyarteritis nodosa (PAN), giant cell arteritis (GCA), and Takayasu's arteritis (TAK), was performed within a set of multicenter longitudinal, observational cohorts. For each form of vasculitis, the frequency of disease-specific manifestations at diagnosis was compared to the cumulative frequency of each manifestation. The percentage of patients who initially developed “severe” manifestations after diagnosis, defined as organ- or life-threatening in the small and medium vessel vasculitides (GPA, MPA, EGPA, PAN) and as ischemic/vascular in the large vessel vasculitides (GCA, TAK), was reported. Results Out of 838 patients with vasculitis, 490 (59%) experienced ≥ 1 new disease manifestation after diagnosis. On average, patients with vasculitis experienced 1.3 new manifestations after diagnosis (GPA - 1.9, MPA - 1.2, EGPA - 1.5, PAN - 1.2, GCA - 0.7, TAK - 1.0). New severe manifestations occurred after diagnosis in 224 (27%) out of 838 patients (GPA - 26%, MPA - 19%, EGPA - 21%, PAN - 23%, GCA - 24%, and TAK - 44%). Timing of onset of new manifestations was not significantly associated with disease duration. Conclusion A majority of patients with vasculitis develop new disease features after diagnosis, including a substantial number of new, severe manifestations. Ongoing assessment of patients with established vasculitis should remain broad in scope. PMID:23908447

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

    Directory of Open Access Journals (Sweden)

    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.

  7. Typical tumors of the petrous bone; Typische Tumoren des Felsenbeins

    Energy Technology Data Exchange (ETDEWEB)

    Ahlhelm, F.; Mueller, U. [Kantonsspital Baden AG, Abteilung fuer Neuroradiologie, Institut fuer Radiologie, Baden (Switzerland); Ulmer, S. [Medizinisch-Radiologisches Institut, Zuerich (Switzerland)

    2014-04-15

    In the region of the petrous bone, inner acoustic canal and cerebellopontine angle, a variety of different tissues can be found, such as bony, epithelial, neural and vascular structures. Tumorous or tumor-like lesions, vascular or bony malformations or other pathologies can therefore be found in all of these areas. We discuss various frequently occurring tumorous or tumor-like pathologies including congential lesions, such as mucoceles, inflammatory disorders including osteomyelitis, pseudotumors and Wegener's granulomatosis. Benign non-neoplastic lesions, such as cholesteatoma, cholesterol granuloma, epidermoid and benign neoplastic tumors, such as the most commonly found vestibular schwannoma, meningeoma, paraganglioma, vascular pathologies and finally malignant lesions, such as metastasis, chordoma or chondrosarcoma and endolymphatic sac tumor (ELST) are also discussed. The emphasis of this article is on the appearance of these entities in computed tomography (CT) and more so magnetic resonance imaging (MRI), it provides key facts and typical images and discusses possibilities how to distinguish these pathologies. (orig.) [German] In der Region des Felsenbein, inneren Gehoerkanals und Kleinhirnbrueckenwinkels findet sich eine Vielzahl an unterschiedlichen Gewebearten inklusive knoechernes, epitheliales, nervales und vaskulaeres Gewebe. Tumoren oder tumoraehnliche Laesionen, ossaere oder vaskulaere Pathologien koennen entsprechend dort gefunden werden. Wir diskutieren verschiedene Tumoren oder tumoraehnliche Pathologien inklusive angeborene Laesionen wie Muko- und Meningozelen, entzuendliche Veraenderungen wie die Osteomyelitis, Pseudotumoren, die Wegener-Granulomatose, nichtneoplastische Tumoren wie das Epidermoid, Cholesteatom oder Cholesterolgranulom und gutartige neoplastische Tumoren wie das am haeufigsten zu findende Vestibularisschwannom, das Paragangliom und das Meningeom, Gefaessprozesse/-pathologien und schliesslich maligne Laesionen wie Metastasen

  8. Chronic respiratory disfunction due to diffuse alveolar hemorrhage in patients with systemic lupus erythematosus and primary vasculitis.

    Science.gov (United States)

    Pérez Aceves, Eva; Pérez Cristóbal, Mario; Espinola Reyna, Gerardo A; Ariza Andraca, Raul; Xibille Fridmann, Daniel; Barile Fabris, Leonor A

    2013-01-01

    Pulmonary hemorrhage (PH) occurs in 2-5% of SLE patients, and is associated with a high mortality rate (79-90%). Diagnostic criteria for this complication include: 1) Pulmonary infiltrates, with at least ¾ of lung tissue involved in a chest x ray, 2) Acute respiratory failure, 3) A decrease of 3g/dL or more in hemoglobin levels. PH might lead to organized pneumonia, collagen deposition, and pulmonary fibrosis which in time might cause changes in pulmonary function tests with either restrictive or obstructive patterns. To evaluate the existence of abnormalities in pulmonary function tests after a PH episode. We included patients with SLE and primary vasculitis that developed PH. During the acute episode, we measured SLEDAI in SLE patients, five factor score in microscopic polyangiitis (MPA) and Birmingham Vasculitis Activity Store (BVAS) in granulomatosis with polyangiitis (GPA) (Wegener). We determined the number of PH events, treatment, and ventilator assistance requirements and correlated its association with abnormal pulmonary function tests. We included 10 patients, 7 with SLE, 2 with MPA and 1 with GPA (Wegener). The mean activity measures were: SLEDAI 20.4 ± 7.5, FFS 2, and BVAS 36. Treatment consisted in methylprednisolone (MPD) in 3 patients, MPD plus cyclophosphamide (CY) in 6 patients, and MPD, CY, IV immunoglobulin, and plasmapheresis in one patient. Five patients required ventilatory support. We found abnormalities in pulmonary function tests in 8 patients, three had an obstructive pattern and five a restrictive pattern; 2 patients did not show any change. We did not find a significant association with any of the studied variables. PH might cause abnormalities in pulmonary function tests and prolonged immunosuppressive treatment could be required. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  9. A Case Report of Arterial Thrombosis in Wegener’s Granulomatosis Presenting with Acute Lower Limb Ischemia

    Directory of Open Access Journals (Sweden)

    Z. Basiri

    2012-04-01

    Full Text Available Introduction: Wegener’s Granolomatosis (WG is a systemic, necrotizing, small-vessel vasculitis. Vascular inflammation and occlusion leading to tissue ischemia is a hallmark of WG. WG has a clinical predilection for the upper airways, lungs, and kidneys. Thromboembolic events do not usually occur and arterial thrombosis is extremely rare.Case Report: Here we reported 2 rare cases of arterial thrombosis that caused lower limb ischemia. There were not any risk factors such as deficiency of protein C, protein S or anti-thrombin 3, Factor V Leiden mutation, and anti-phospholipids syndrome. Limb perfusion returned as a result of emergency treatment and ischemia did not occur. High doses of prednisolone and endoxan were administrated for them. Conclusion: The thrombosis seemed to happen due to the inflammation process of the disease itself. Because of possible morbidity of limb gangrene we suggest special notice to limb pain, evaluation by paraclinics such as color doppler sonography or angiography to rule out or rule in thromboembolism, determining whether there are risk factors for thrombosis such as (deficiency of protein C and protein S or anti-thrombin III, Leiden 5 factor mutation and anti-phospholipid antibody syndrome, and treatment or removal of them. If no risk factor is found, high doses of immunosuppressive therapy like steroid and cytotoxic agents like Endoxan will be the choice.(Sci J Hamadan Univ Med Sci 2012;19(1:75-78

  10. Granulomatous peritonitis due to Ascaris lumbricoides. Case report Granulomatosis peritoneal por Ascaris lumbricoides.Presentación de un caso

    Directory of Open Access Journals (Sweden)

    Carlos Aguirre Muñoz

    2008-06-01

    Full Text Available

    Ascariasis is one of the most prevalent and widespread human helminthic infections, especially in children of the underdeveloped countries. It is related to conditions of poverty, malnutrition and poor hygiene. According to the complex life cycle of the parasite, several and different intestinal and extraintestinal complications can arise, including peritonitis. Appropriate treatment and preventive measures are very important factors to avoid deaths caused by this disease.

    We report the case of a 3 year-old girl, coming from a rural area of Colombia, who presented peritonitis by Ascaris lumbricoides, preceded by persistent diarrhea, pain and abdominal mass. She was incidentally diagnosed by means of laparotomy and peritoneal biopsy, because an intestinal lymphoma was suspected.

    La ascariasis es una parasitosis ampliamente diseminada en el mundo, especialmente en los niños de los países en vías de desarrollo, ligada a condiciones de pobreza, desnutrición y mala higiene. Se acepta que es la geohelmintiasis más frecuente entre todas las parasitosis humanas. Dado el complejo ciclo de vida del parásito causal, se pueden producir muchas y variadas complicaciones intestinales y extraintestinales, entre ellas la peritonitis. El tratamiento oportuno y las medidas preventivas son factores importantísimos para evitar las muertes por esta enfermedad.

    Se presenta el caso de una niña de 3 años de edad, procedente de una zona rural colombiana quien presentó una peritonitis por áscaris, manifestada por diarrea persistente, dolor y masa abdominal, en quien se hizo el diagnóstico en forma casual mediante laparotomía y biopsia peritoneal, ante la sospecha de un linfoma.

  11. STAT4 gene polymorphism is associated with psoriasis in the genetically homogeneous population of Crete, Greece.

    Science.gov (United States)

    Zervou, Maria I; Goulielmos, George N; Castro-Giner, Francesc; Tosca, Androniki D; Krueger-Krasagakis, Sabine

    2009-09-01

    Recent genome-wide association studies (GWAS) of many complex diseases have successfully identified novel susceptibility loci, with many of them being associated with more than one condition. Taking into consideration that different autoimmune diseases may share some common pathogenetic pathways, we hypothesized that STAT4, a susceptibility gene found to be associated with increased risk for systemic lupus erythematosus, rheumatoid arthritis, type 1 diabetes, Sjögren's syndrome, Wegener's granulomatosis, Crohn's disease, and ulcerative colitis may also have a role in psoriasis. Psoriasis is an autoimmune, chronic inflammatory skin disease. Here we performed a case-control study in the population of island of Crete and demonstrated for the first time the association of a STAT4 single nucleotide polymorphism (SNP) with susceptibility to psoriasis, thus suggesting a putative key role of STAT4 in multiple autoimmune diseases. We found that mutated allele T of the STAT4 rs7574865 SNP, which previously was implicated in the predisposition to many autoimmune diseases, were more common in individuals with psoriasis than in controls (p = 0.045, odds ratio = 1.42, 95% confidence interval 1.01-2.00), thus concluding that the polymorphism examined is associated with the development of psoriasis in our population.

  12. "One-stop shop" spectral imaging for rapid on-site diagnosis of lung cancer: a future concept in nano-oncology.

    Science.gov (United States)

    Darwiche, Kaid; Zarogoulidis, Paul; Krauss, Leslie; Oezkan, Filiz; Walter, Robert Fred Henry; Werner, Robert; Theegarten, Dirk; Sakkas, Leonidas; Sakkas, Antonios; Hohenforst-Scmidt, Wolfgang; Zarogoulidis, Konstantinos; Freitag, Lutz

    2013-01-01

    There are currently many techniques and devices available for the diagnosis of lung cancer. However, rapid on-site diagnosis is essential for early-stage lung cancer, and in the current work we investigated a new diagnostic illumination nanotechnology. Tissue samples were obtained from lymph nodes, cancerous tissue, and abnormal intrapulmonary lesions at our interventional pulmonary suites. The following diagnostic techniques were used to obtain the samples: endobronchial ultrasound bronchoscopy; flexible bronchoscopy; and rigid bronchoscopy. Flexible and rigid forceps were used because several of the patients were intubated using a rigid bronchoscope. In total, 30 tissue specimens from 30 patients were prepared. CytoViva® illumination nanotechnology was subsequently applied to each of the biopsy tissue slides. A spectral library was created for adenocarcinoma, epidermal growth factor receptor mutation-positive adenocarcinoma, squamous cell carcinoma, usual interstitial pneumonitis, non-specific interstitial pneumonitis, typical carcinoid tumor, sarcoidosis, idiopathic pulmonary fibrosis, small cell neuroendocrine carcinoma, thymoma, epithelioid and sarcomatoid mesothelioma, cryptogenic organizing pneumonia, malt cell lymphoma, and Wegener's granulomatosis. The CytoViva software, once it had created a specific spectral library for each entity, was able to identify the same disease again in subsequent paired sets of slides of the same disease. Further evaluation of this technique could make this illumination nanotechnology an efficient rapid on-site diagnostic tool.

  13. [Recurrent new-onset uveitis in a patient with rheumatoid arthritis during anti-TNFalpha treatment].

    Science.gov (United States)

    Di Gangi, M; Foti, R; Leonardi, R; Leonetti, C; Castellino, P

    2007-01-01

    Inflammation involving the uveal tract of the eye, termed uveitis, is frequently associated with various rheumatic disease, including seronegative spondylarthropathies, juvenile rheumatoid arthritis, Crohn's disease and Behçet's disease. Scleritis and keratitis may be associated with rheumatoid arthritis and systemic vasculitides such as Wegener's granulomatosis. Immune-mediated uveitis can have a chronic relapsing course and produce numerous possible complications, many of which can result in permanent vision loss. Treatment typically includes topical or systemic corticosteroids with cycloplegic-mydriatic drugs and/or noncorticosteroid immunosuppressants, but often there is an insufficient clinical effectiveness. Anti-TNFalpha therapy is promising in the treatment of sight threatening uveitis, particularly in patients with Behçet's disease. However, there have been also reports of new-onset uveitis during treatment of joint disease with TNFalpha inhibitors. We describe a case of new-onset uveitis in a patient with rheumatoid arthritis during therapy with etanercept at first and infliximab at last. Although we cannot exclude uveitis as linked to rheumatoid arthritis, it is unlike that the uveitis arises when the joint disease is well controlled. The hypothetical paradoxical effect of anti-TNF is here discussed.

  14. Mathematical analysis of antigen selection in somatically mutated immunoglobulin genes associated with autoimmunity.

    Science.gov (United States)

    MacDonald, C M; Boursier, L; D'Cruz, D P; Dunn-Walters, D K; Spencer, J

    2010-09-01

    Affinity maturation is a process by which low-affinity antibodies are transformed into highly specific antibodies in germinal centres. This process occurs by hypermutation of immunoglobulin heavy chain variable (IgH V) region genes followed by selection for high-affinity variants. It has been proposed that statistical tests can identify affinity maturation and antigen selection by analysing the frequency of replacement and silent mutations in the complementarity determining regions (CDRs) that contact antigen and the framework regions (FRs) that encode structural integrity. In this study three different methods that have been proposed for detecting selection: the binomial test, the multinomial test and the focused binomial test, have been assessed for their reliability and ability to detect selection in human IgH V genes. We observe first that no statistical test is able to identify selection in the CDR antigen-binding sites, second that tests can reliably detect selection in the FR and third that antibodies from nasal biopsies from patients with Wegener's granulomatosis and pathogenic antibodies from systemic lupus erythematosus do not appear to be as stringently selected for structural integrity as other groups of functional sequences.

  15. Hemorrhagic cystitis; an old story with new advancements

    Directory of Open Access Journals (Sweden)

    Ibrahim Yildirim

    2012-04-01

    Full Text Available Cyclophosphamide (CP, an oxazaphosphorine alkylating agent introduced in 1958, is widely used in the treatment of solid tumors and B-cell malignant disease, such as lymphoma, myeloma, chronic lymphocytic leukemia and Waldenstrom macroglobulinemia. Furthermore, CP and ifosfamide, a synthetic analogue of CP, have had an increasing role in the treatment of nonneoplastic diseases, such as thrombocytopenic purpura, rheumatoid arthritis, systemic lupus erythematosis, nephritic syndrome, and Wegener granulomatosis, and as a conditioner before bone marrow transplantation. Soon after their discovery, as early as 1960, the first side-effects of CP were reported by Coggins and co-workers. These side-effects have been reported as transient irritative voiding symptoms, including urinary frequency, dysuria, urgency, suprapubic discomfort and strangury with microhematuria as well as life-threatening hemorrhagic cystitis. Bladder fibrosis, necrosis, contracture, and vesicoureteral reflux also have been reported. Hemorrhage usually occurs during or immediately after treatment, whether with short-term high or long-term low dosages. In 1981, Brock et al have documented that... [J Exp Integr Med 2012; 2(2.000: 93-94

  16. Long-term follow-up of different refractory systemic vasculitides treated with rituximab.

    Science.gov (United States)

    Rees, Frances; Yazdani, Ramin; Lanyon, Peter

    2011-09-01

    There is increasing interest in rituximab (RTX) as an alternative to cyclophosphamide (CYC) for remission induction in systemic vasculitis. Recent studies have reported high remission rates, but it is not clear how long the initial remission lasts [1, 2]. A retrospective study was undertaken of 15 cases of refractory systemic vasculitis (11 Wegener's granulomatosis, 1 Churg-Strauss syndrome, 1 cutaneous polyarteritis nodosa and 2 cryoglobulinaemic vasculitis) treated with RTX, with a mean follow-up of 34 months. All had previously received CYC, and 14, at least one other immunosuppressive drug. All had active disease when treated (median Birmingham Vasculitis Activity Score (BVAS) 2003, 13). All cases achieved remission (BVAS 2003, 0). Thirteen required re-treatment, nine due to relapse (mean, 9 months after initial treatment) and four because of repopulation or rising ANCA in the context of CYC intolerance or previous CYC refractory disease. Relapsing cases have been successfully re-treated up to five further cycles, either at B cell repopulation or at six monthly intervals. Infections were rare. Mean IgG levels fell significantly, and IgM levels became subnormal in six cases. There were three cases of neutropenia, one severe at 10 months post-treatment. These results provide further evidence that RTX is an effective induction agent in systemic vasculitis. The optimal and long-term outcome of re-treatment remains to be defined.

  17. Lung vasculitis and alveolar hemorrhage: pathology.

    Science.gov (United States)

    Fishbein, Gregory A; Fishbein, Michael C

    2011-06-01

    Pulmonary vasculitides are a diverse group of limited and systemic disorders associated with inflammation of pulmonary vessels and parenchyma. These diseases often have distinctive clinical, serological, and histopathological features-extrapulmonary sites of involvement, circulating autoantibodies, predispositions for small or large vessels, and others. Some have characteristic inflammatory lesions; others are characterized by the absence of such lesions. Frequently pathological findings overlap, rendering classification, and diagnosis a challenge. The anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel diseases constitute the major pulmonary vasculitides. These include Wegener granulomatosis (WG), Churg Strauss syndrome (CSS), and microscopic polyangiitis (MPA). Less frequently, diseases such as polyarteritis nodosa, Takayasu arteritis, Behçet syndrome, and connective tissue diseases may involve pulmonary vessels, but these entities are better associated with extrapulmonary disease. Diffuse alveolar hemorrhage (DAH) is a severe manifestation of pulmonary vasculitis. DAH is most commonly seen in small-vessel vasculitides, specifically MPA and WG. Other syndromes associated with DAH include Goodpasture syndrome, Henoch-Schönlein purpura, and systemic lupus erythematosus. Less commonly, DAH may be secondary to infection or drugs/toxins. Furthermore, in the absence of discernable systemic disease, DAH may be idiopathic-referred to as isolated pulmonary capillaritis (IPC) or idiopathic pulmonary hemosiderosis (IPH), depending on the presence of capillaritis.

  18. 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.

  19. 原发性小血管炎的早期诊断和治疗

    Institute of Scientific and Technical Information of China (English)

    连希艳; 黄胜华

    2010-01-01

    @@ 原发性小血管炎(primary small vessel vasculitis,PSV)是一组主要累及小血管(毛细血管、小静脉、微小动脉等),以血管壁坏死性炎性反应、纤维素样坏死为病理特征的一类自身免疫性疾病,包括显微镜下多血管炎(microscopic polyangiitis,MPA)、韦格纳肉芽肿(Wegener granulomatosis,WG)、过敏性肉芽肿性血管炎(Churg-Strauss syndrome,CSS)、抗中性粒细胞胞质抗体(ANCA)相关性坏死性新月体性肾炎(necrotizing crescentic glomerulonephritis,NCGN)[1],因其相当部分与ANCA关系密切,因而也称为ANCA相关性小血管炎(ANCA associated systemic vasculitis,AASV).

  20. Current progress in the immunopathogenesis of ANCA-associated systemic vasculitis%ANCA相关性血管炎的免疫发病机制新进展

    Institute of Scientific and Technical Information of China (English)

    唐莎; 张静波

    2013-01-01

    Antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV) are a series of autoimmune disorders including Wegener granulomatosis (WG),microscopic polyangiitis (MPA),Churg-Strauss syndrome (CSS) and renal-limited vasculitis (RLV).Evidence from clinical studies and experiments has illustrated the pathogenic role of ANCA in the development of AASV.The present review will cover the fundamental role and highlight the latest progress of neutrophils,effector T cells,infection and molecular mimicry,pathogen-associated molecular patterns (PAMPs),complement system as well as tertiary lymphoid tissue involved in AASV pathogenesis.%抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibodies,ANCA)相关性系统性血管炎(ANCA-associated systemic vasculitis,AASV)是典型的自身免疫疾病,其发病机制包括天然免疫和获得性免疫的共同参与.大量临床及实验证据显示ANCA不仅是AASV的血清学标志,也在血管及组织损伤中起核心作用.本文就ANCA作用模式、感染与分子模拟、病原体相关分子模式识别、补体系统及三级淋巴组织参与免疫发病机制的最新进展作一综述.

  1. Systemic and non-systemic vasculitis affecting the peripheral nerves.

    Science.gov (United States)

    Finsterer, J

    2009-06-01

    Vasculitis affecting the peripheral nerves predominantly manifests as subacute, progressive, asymmetric sensorimotor polyneuropathy or mononeuritis multiplex, and more rarely as painful mononeuropathy, pure sensory neuropathy, neuropathy of the cranial nerves, plexopathy, or as autonomic neuropathy. Vasculitic neuropathy may occur isolated or non-isolated (systemic) together with involvement of other organs. Systemic vasculitis with involvement of the peripheral nerves is further subdivided into primary (Takayasu syndrome, giant cell arteritis, classical panarteritis nodosa, thrombangitis obliterans, Kawasaki disease, Churg-Strauss syndrome, Wegener granulomatosis, cryoglobulinemic vasculitis, Behcet disease, microscopic polyangitis, Schoenlein Henoch purpura) or secondary systemic vasculitis (autoimmune connective tissue diseases, vasculitis from infection, sarcoidosis, malignancy, drugs, radiation, or diabetes). In addition to routine laboratory investigations and nerve conduction studies, nerve biopsy is essential for diagnosing the condition and to delineate it from differentials, although its sensitivity is only approximately 60%. Therapy of non-viral vasculitic neuropathy is based on corticosteroids and cyclophosphamide alone or in combination. Additional options include azathioprine, methotrexate, mycophenolate mofetil, or rituximab. In single cases immunoglobulins, immunoadsorbtion, or plasma exchange have been successfully applied. In case of virus-associated vasculitis interferon-alpha plus lamivudine or ribaverin may be beneficial.

  2. Collagenolytic (necrobiotic) granulomas: part II--the 'red' granulomas.

    Science.gov (United States)

    Lynch, Jane M; Barrett, Terry L

    2004-07-01

    A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. 'blue' granulomas vs. 'red' granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In the previously published first part, the clinical presentation, pathogenesis and histologic features of the 'blue' collagenolytic granulomas were discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In this second half of the series, the 'red' collagenolytic granulomas are discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's Syndrome).

  3. [Ocular manifestations of rheumatic diseases. Cooperation between internist/ophthalmologist].

    Science.gov (United States)

    Tyndall, A; Steiger, U

    1993-05-01

    A red or painful eye may be the clue to a systemic condition, many of which are of a rheumatological or immunological nature. Conjunctivitis may occur in Sjögren's Syndrome, Reiter's Syndrome (and other sero negative spondyloarthropathies) and with infections such as chlamydia and viruses. 70% of cases of episcleritis are idiopathic, the other 30% being associated with rheumatoid arthritis or other connective tissue diseases or herpes zoster infection. Scleritis may be seen with connective tissue diseases or auto immune conditions (rheumatoid arthritis, Wegener granulomatosis, polyarteritis nodosa, relapsing polychondritis, SLE), infections (herpes, tuberculosis, syphilis, aspergillosis) or metabolic (gout, porphyria, cystinosis). Retinal vasculitis is seen in SLE, Behçet's Disease, sarcoidosis, polyarteritis nodosa, Whipple's disease and Crohn's disease among others. However, uveitis presents perhaps the greatest diagnostic challenge and interface between ophthalmology and rheumatology. Some syndromes are purely ophthalmological (eg: Fuch's heterochromic cyclitis) but others may lead to the diagnosis of a rheumatic disorder (eg: recurrent unilateral acute anterior uveitis and ankylosing spondylitis). Systemic syndromes most likely to be associated with uveitis are Reiter's disease, ankylosing spondylitis, sarcoidosis, juvenile arthritis, interstitial nephritis, inflammatory bowel disease, syphilis. The patterns are different, eg: acute painful unilateral anterior uveitis with ankylosing spondylitis and chronic asymptomatic bilateral uveitis in juvenile arthritis (ANA positive, pauci-articular) or bilateral symptomatic uveitis in sarcoidosis. An illustrative case will be presented and an algorithm for the evaluation of uveitis discussed.

  4. Biplane CT of intraorbital and intracranial complicated lesions of nasal origin

    Energy Technology Data Exchange (ETDEWEB)

    Sadamoto, K.; Sadamoto, M. (Sadamoto Hospital of the Washo-Kai Medical Foundation, Ehime (Japan)); Koike, S.

    1981-12-01

    Thirty-three patients with intraorbital and intracranial complicated lesions of nasal origin were examined by means of the biplane CT (combination of axial and coronal CT) technique. The cases were comprised of 1 case of orbital cellulitis, 1 case of a postoperative maxillary cyst, 6 cases of mucocele (or pyocele), 2 cases of progressive gangrenous rhinitis (including Wegener's granulomatosis), and 23 cases of maxillary cancer. In the case of orbital cellulitis, a markedly dilated superior ophthalmic vein was observed by biplane CT for the first time. A remarkable swelling of the superior rectus and levator palpebral superior muscles was confirmed precisely by coronal CT. In the 6 cases of mucocele (or pyocele), coronal CT scans confirmed the original lesions from the frontal sinus or from the ethmoid sinus. In the 23 cases of maxillary cancer, coronal CT was especially useful for the observation of the precise infiltration in the intraorbit, base of the skull, intracranium, and subtemporal fossa. It is concluded that this technique, i.e., biplane CT, is reliable for the diagnosis of these lesions of nasal origin and is useful for the TNM-classification of UICC.

  5. Bone marrow accumulation in gallium scintigraphy in patients with adult still's disease

    Energy Technology Data Exchange (ETDEWEB)

    Kanegae, Futoshi; Tada, Yoshifumi; Ohta, Akihide; Ushiyama, Osamu; Suzuki; Noriaki; Koarada, Syuichi; Haruta, Yoshio; Yoshikai, Tomonori; Nagasawa, Kohei [Saga Medical School (Japan)

    2002-12-01

    We investigated the features and the usefulness of gallium scintigraphy in the diagnosis and the assessment of Adult Still's disease (ASD) by retrospective case review. Gallium scintigraphy have been done for 11 cases of ASD (3 males and 8 females) and 4 females were positive. Among these, 67 Ga-citrate was accumulated to the bone marrow in all 4 cases and to the major joints in 2 cases. Positive cases were rather serious and administered more immunosuppressants than negative cases. In order to characterize gallium scintigraphy findings of ASD, i.e. bone marrow accumulation, we analyzed 130 cases of collagen vascular disease. Although 101 cases (77.7%) were positive, only 7 cases (5.4%) showed the accumulation of {sup 67}Ga-citrate to the bone marrow. These include 3 cases with ASD, and 1 case with systemic lupus erythematosus, polyarteritis nodosa, Wegener's granulomatosis and Sjogren's syndrome. We also accumulated 18 patients who exhibited bone marrow accumulation of {sup 69}Ga-citrate, and found that 7 patients had collagen vascular and their related diseases. In conclusion, bone marrow accumulation in gallium scintigraphy is a specific feature of collagen vascular diseases, especially ASD, and it is suggested that cases with positive gallium scintigraphy in ASD can be serious and resistant to treatment. (author)

  6. Manifestações otorrinolaringológicas nas doenças reumáticas auto-imunes Otorhinolaryngologic manifestations of autoimmune rheumatic diseases

    Directory of Open Access Journals (Sweden)

    Daniela Bergamim Pereira

    2006-04-01

    systemic lupus erythematosus, Wegener's granulomatosis, relapsing polychondritis, polyarteritis nodosa, Cogan's syndrome, Sjögren's syndrome, and less frequently in Churg-Strauss syndrome and Behçet's disease. Nose and paranasal sinuses are variably affected during the course of Wegener's granulomatosis, Churg-Strauss syndrome, relapsing polychondritis and sarcoidosis. Recurrent mucosal ulcerations are common in systemic lupus erythematosus and Behçet's disease. Xerostomia is a common feature of primary and secondary Sjögren's syndrome; salivary gland enlargement may be also seen in these patients, as well as in patients with sarcoidosis. The cricoarytenoid joint can be involved during the course of rheumatoid arthritis, ankylosing spondylitis and gout; osteoarthritic changes have also been described. Trigeminal nerve dysfunction may occur in patients with Sjögren's syndrome, systemic sclerosis, systemic lupus erythematosus and mixed connective tissue disease. Peripheral facial nerve palsy has been described to complicate the course of Sjögren's syndrome and sarcoidosis.

  7. Ongevallen met langzaam verkeer en zwaar verkeer op wegen met een snelheidslimiet van 50 of 80 km/uur : aanzet tot aanvullende veiligheidscriteria voor een Kwaliteitsnet Goederenvervoer.

    NARCIS (Netherlands)

    Dijkstra, A.

    2009-01-01

    Verschillende Nederlandse overheden streven naar kwaliteits-netten voor goederenvervoer, dat wil zeggen routes waarlangs zwaar verkeer bij voorkeur zou moeten rijden. Er zijn diverse criteria opgesteld waar de kwaliteitsnetten aan moeten voldoen. Dit rapport draagt bij aan een verdere uitbreiding va

  8. Snelheidsbeheersing op 50 km/uur-wegen. Bijdrage aan `Gemeente-info' van september/oktbober 1995, met betrekking tot het speerpunt `Aangepaste snelheden'.

    OpenAIRE

    Catshoek, J.W.D.

    1996-01-01

    In this report, speed control on 50 km/h roads in the Netherlands is dealt with. The report includes the following: a description of the speed problem; an explanation of the importance of structural measurements (monitoring) of driving speeds on main roads; a design for simple speed measurements; and example projects.

  9. Borgen van verkeersveiligheid bij het aanbesteden van wegen : review van de aanbesteding van het RWS Zuid-Holland-project A4 Burgerveen-Leiden.

    NARCIS (Netherlands)

    Schermers, G. Wesemann, P. & Stipdonk, H.L.

    2008-01-01

    The Division Zuid-Holland of the Directorate-General for Public Works and Water Management has used a new procedure for contracting the widening of the A4 motorway near Leiden. The new procedure paid special attention to road safety in the framework of functional contracting. Tenderers could receive

  10. Tussen tellen en wegen : over de ontwikkeling van de instellingsbeoordeling door de commissies Theater, Jeugdtheater, Dans en Productiehuizen van de Raad voor Cultuur

    NARCIS (Netherlands)

    van den Hoogen, Quirijn

    2016-01-01

    This document reports on the outcomes of a study on the evaluation of subsidy applications by the Dutch Council for Culture. Undeniably, the assessment has become more systematic over the years. Scores are being given to the various aspects that need to be evaluated. Evaluations of subsidy applicati

  11. Aanwezigheid en gebruik van autogordels 1985. Verslag van waarnemingen gedaan bij bestuurders en voorpassagiers van personenauto's op wegen binnen en buiten de bebouwde kom.

    NARCIS (Netherlands)

    Arnoldus, J.G & Scholtens, H.P.

    1986-01-01

    Using a method designed in 1978, whereby an observer looks inside the car while it is waiting at traffic lights to see whether belts are being worn, the development of seat belt usage in the Netherlands during 1985 is described and analyzes. See also PB 24546 for usage in 1984,

  12. Chronic granulomatous disease

    Science.gov (United States)

    CGD; Fatal granulomatosis of childhood; Chronic granulomatous disease of childhood; Progressive septic granulomatosis ... In chronic granulomatous disease (CGD), immune system cells called phagocytes are unable to kill some types of bacteria and ...

  13. Clinical Transcriptomics in Systemic Vasculitis (CUTIS)

    Science.gov (United States)

    2016-12-22

    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

  14. Espacios aéreos pulmonares anormales por TCAR Abnormal air-filled spaces in the lung with HRCT

    Directory of Open Access Journals (Sweden)

    Juan Carlos Spina (h

    2008-06-01

    Full Text Available Objetivo: El propósito de este trabajo es revisar y dar claves para el diagnóstico de las distintas entidades que se presentan como espacios aéreos pulmonares anormales. Material y métodos: Se analizaron en forma retrospectiva los archivos de TCAR de nuestra institución con diagnóstico de espacios aéreos pulmonares anormales. En todos los casos se evaluó la localización, número (único o múltiples, grosor parietal y alteraciones parenquimatosas y mediastínicas asociadas y se los agrupo de acuerdo con su asociación con distintas enfermedades. Resultados: En base a las características topográficas, los espacios aéreos anormales fueron agrupados en: etiología infecciosa (tuberculosis, neumonía por Pneumocistis jiroveci, aspergilosis, hidatidosis, neumonía cavitada, absceso y neumatocele, enfermedades que producen bronquiectasias (fibrosis quística, síndrome de Kartagener, aspergilosis broncopulmonar alérgica, vasculitis ( granulomatosis de wegener y artritis reumatoidea, enfisema, neoplasias no primitivas (metástasis cavitadas, tumores primarios (carcinoma epidermoide y bronquioloalveolar y otras (linfangioleiomiomatosis, histiocitosis de células de Langerhans, secuestro pulmonar y malformación adenoidea quística. Conclusión: Los espacios aéreos pulmonares anormales son un hallazgo frecuente en los estudios por TCAR. Teniendo en cuenta cierta característica de los mismos, es posible arribar a un diagnóstico probable en la mayoría de los casos.Objetive: The purpose of this paper is to review and give some clues for the diagnosis of the many entities that present as abnormal air spaces at HRCT. Materials and methods: We retrospectively reviewed the archives of HRCT of our institution with diagnosis of different entities that presented with abnormal air spaces. In all cases we evaluated the location, number (unique o multiple, parietal thickness and concomitant compromise of parenchyma or mediastinum and we clustered

  15. Características generales de 29 pacientes con vasculitis de pequeños vasos General characteristics of 29 patients with small vessel vasculitis

    Directory of Open Access Journals (Sweden)

    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

  16. 19例口面部肉芽肿病临床及组织病理分析%Clinicopathologic analysis of orofacial granulomatosis: a 19-case report

    Institute of Scientific and Technical Information of China (English)

    储召娓; 张欢; 彭斌; 杨元元; 耿松梅

    2016-01-01

    目的:探讨口面部肉芽肿病(OFG)临床、组织病理特征及局部肿胀的机制.方法:对19例OFG患者的临床和组织病理资料进行回顾性分析,并进行CD68、CD31和D2-40免疫组化染色.结果:19例OFG患者中,男7例,女12例,年龄13~76岁,平均50.32岁.皮损以唇最常见,占100%,表现为持续性非凹陷性浸润性肿胀和红斑.OFG组织病理特征为真皮内上皮样肉芽肿形成,中心无坏死.17例患者(89.47%)可见肉芽肿围绕D2-40阳性的淋巴管形成,13例患者(68.42%)可见肉芽肿位于D2-40阳性的淋巴管内.结论:OFG可侵犯面部多部位,上皮样肉芽肿是其主要组织病理特征,淋巴管阻塞及淋巴引流障碍是其局部肿胀的病理机制.

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

    Directory of Open Access Journals (Sweden)

    María Virginia Paolini

    2013-04-01

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

  18. The Clinical Course of Late Diagnosed Fatal Cases of A (H1N1 Influenza in Poland 

    Directory of Open Access Journals (Sweden)

    Marta Rorat

    2013-06-01

    Full Text Available Introduction: The most frequent complication of A (H1N1 influenza and the leading cause of death was pneumonia with a primary viral or mixed viral and bacterial etiology. 182 patients had died because of a pandemic influenza in Poland by 31st July 2010.Material and Methods: A retrospective study of 6 fatal cases of pandemic influenza, aged 23-41, including 3 women, hospitalised between November 2009 and February 2011 in different Polish medical centres.Results: We present the clinical course of 6 late diagnosed cases of A (H1N1 influenza. All patients presented typical flu-like symptoms in the beginning. 4/6 patients had severe disease risk factors: pregnancy, arthritis, Wegener granulomatosis and obesity. All patients were seen by doctors, no one had received antiviral therapy, 4/5 were treated with antibiotics before they were hospitalized. One patient had nosocomial infection. Patients were admitted to the hospital on the 3rd to 8th day of the disease. They received oseltamivir treatment on the 4th to 9th day. All patients developed pneumonia complicated by acute respiratory distress syndrome. Death appeared between the 4th and 27th day after the onset of symptoms. Autopsies were performed in 5 cases and revealed haemorrhagic pneumonia in 2 patients.Conclusion: Delayed diagnosis and antiviral treatment initiation has a significant impact on mortality in A (H1N1 influenza. During the influenza epidemic, patients presenting typical symptoms should always be suspected of having influenza. Antiviral treatment has to be initiated immediately, especially ifthere are risk factors of severe disease.

  19. Transmembrane TNF-alpha reverse signaling leading to TGF-beta production is selectively activated by TNF targeting molecules: Therapeutic implications.

    Science.gov (United States)

    Szondy, Zsuzsa; Pallai, Anna

    2017-01-01

    Tumor necrosis factor (TNF)-α is a potent pro-inflammatory cytokine exerting pleiotropic effects on various cell types. It is synthesized in a precursor form called transmembrane TNF-α (mTNF-α) which, after being processed by metalloproteinases, is released in a soluble form to mediate its biological activities through Type 1 and 2 TNF receptors in TNF receptor expressing cells. In addition to acting in soluble form, TNF-α also acts in the transmembrane form both as a ligand by activating TNF receptors, as well as a receptor that transmits outside-to-inside (reverse) signals back into mTNF-α bearing cells. Since the discovery that TNF-α plays a determining role in the pathogenesis of several chronic inflammatory diseases, anti-TNF agents are increasingly being used in the treatment of a rapidly expanding number of rheumatic and systemic autoimmune diseases, such as rheumatoid arthritis, Crohn's disease, psoriasis, psoriatic arthritis, ankyloting spondylitis, Wegener granulomatosis and sarcoidosis. There are 5 TNF antagonists currently available: etanercept, a soluble TNF receptor construct; infliximab, a chimeric monoclonal antibody; adalimumab and golimumab, fully human antibodies; and certolizumab pegol, an Fab' fragment of a humanized anti-TNF-α antibody. Though each compound can efficiently neutralize TNF-α, increasing evidence suggests that they show different efficacy in the treatment of these diseases. These observations indicate that in addition to neutralizing TNF-α, other biological effects induced by TNF-α targeting molecules dictate the success of the therapy. Recently, we found that mTNF-α reverse signaling leads to transforming growth factor (TGF)-β production in macrophages and anti-TNF agents selectively trigger this pathway. In this review we will focus on the potential contribution of the activation of the mTNF-α signaling pathway to the success of the anti-TNF therapy.

  20. Development and validation of case-finding algorithms for the identification of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis in large healthcare administrative databases.

    Science.gov (United States)

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

    2016-12-01

    The aim of this study was to develop and validate case-finding algorithms for granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (Churg-Strauss, EGPA). Two hundred fifty patients per disease were randomly selected from two large healthcare systems using the International Classification of Diseases version 9 (ICD9) codes for GPA/EGPA (446.4) and MPA (446.0). Sixteen case-finding algorithms were constructed using a combination of ICD9 code, encounter type (inpatient or outpatient), physician specialty, use of immunosuppressive medications, and the anti-neutrophil cytoplasmic antibody type. Algorithms with the highest average positive predictive value (PPV) were validated in a third healthcare system. An algorithm excluding patients with eosinophilia or asthma and including the encounter type and physician specialty had the highest PPV for GPA (92.4%). An algorithm including patients with eosinophilia and asthma and the physician specialty had the highest PPV for EGPA (100%). An algorithm including patients with one of the diagnoses (alveolar hemorrhage, interstitial lung disease, glomerulonephritis, and acute or chronic kidney disease), encounter type, physician specialty, and immunosuppressive medications had the highest PPV for MPA (76.2%). When validated in a third healthcare system, these algorithms had high PPV (85.9% for GPA, 85.7% for EGPA, and 61.5% for MPA). Adding the anti-neutrophil cytoplasmic antibody type increased the PPV to 94.4%, 100%, and 81.2% for GPA, EGPA, and MPA, respectively. Case-finding algorithms accurately identify patients with GPA, EGPA, and MPA in administrative databases. These algorithms can be used to assemble population-based cohorts and facilitate future research in epidemiology, drug safety, and comparative effectiveness. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  1. 477 Autoimmunity in Patients with Allergy and Immunodeficiency: as Result of the Immune Chronic Inflammation

    Science.gov (United States)

    Mendez-Inocencio, Julia; Bellanti, Joseph

    2012-01-01

    Background Autoimmunity is present in several patients that have allergy and immunodeficiency; however there are few reports that correlate these 3 immunological problems. Our objective was to study the association of these 3 problems where the deregulation of the T reg cells has a roll in triggering a chronic inflammation and the clinical expression. Methods We included 8 patients with symptoms of autoimmune disease, such as autoimmune hypothyroidism, dermatomiositis, systemic vasculitis, autoimmune uveitis, Wegener´s granulomatosis, antiphospholipid syndrome, Kawasaki´s disease and SLE with allergic disease and T and B lymphocytes immunodeficiency. We did the clinical history, skin test and also immunological evaluation with immunoglobulins, IgG subclasses, T lymphocytes absolute numbers and specific antibodies, all of them had diagnosis of allergy, with immunodeficiency and autoimmune disease. Results There were included 8 patients, with moderate to severe allergy and recurrent infections, from 3 to 66 years of age, 1 child (12.5%) and 7 adults (87.5%), 7 women (93.3%), 1 men (12.5%) with allergic rhinitis 8 (100%), combination of allergic rhinitis and other allergic disease 2 (25%), asthma 1 (12.5%), atopic dermatitis 1 (12.5%). Also all the 8 patients had humoral, cellular immunodeficiency and autoimmune problems, 7 of them received IVIG therapy on the basis of the immunodeficiency, with evident improvement, 1 did not received and had some improvement of their symptoms. Conclusions We found out that the 8 patients with autoimmunity had allergy and also mixed T and B immunodeficiency. We conclude that the physicians should be aware of the correlation in chronic inflammation and the presence of the 3 immunological clinical problems to give appropriate treatment and improve the prognosis of these patients.

  2. Genetic aspects of anti-neutrophil cytoplasmic antibody-associated vasculitis.

    Science.gov (United States)

    Alberici, Federico; Martorana, Davide; Vaglio, Augusto

    2015-04-01

    The genetics of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complex area of investigation because of the low frequency of AAVs, the rarity of familial cases and the complexity of disease phenotypes. However, recent studies have been able to gather significant numbers of patients, and multicentre collaborative efforts have allowed the performance of two genome-wide association studies (GWASs). Genetic association studies based on candidate gene approaches and the two GWASs have greatly contributed to our current understanding of the genetic basis of AAV. The central role of autoimmunity has been confirmed by the significant association with HLA polymorphisms; interestingly, the three main AAV subtypes are associated with distinct HLA variants, i.e. granulomatosis with polyangiitis (Wegener's GPA) with HLA-DP1, microscopic polyangiitis with HLA-DQ and eosinophilic GPA (Churg-Strauss) with HLA-DRB4. GWASs also revealed that polymorphic variants of genes encoding proteinase 3 (PR3), the predominant antigenic target of ANCA in GPA, and its main inhibitor, alpha-1 antitrypsin, are highly associated with GPA and, even more significantly, with PR3-ANCA positivity (regardless of the clinical diagnosis); this emphasizes the central pathogenic role of PR3 and humoral autoimmunity in PR3-ANCA positive vasculitis. Finally, candidate gene approach studies have shown associations with other variants involved in autoimmunity, such as those belonging to the CTLA-4 and PTPN22 genes, although these findings warrant replication in larger studies. Additional studies are underway to better characterize disease associations within the AAV spectrum, which could provide new pathogenetic clues and possibly new treatment targets.

  3. Crescentic glomerulonephritis in children.

    Science.gov (United States)

    Jardim, H M; Leake, J; Risdon, R A; Barratt, T M; Dillon, M J

    1992-05-01

    Data on patients with crescentic glomerulonephritis (greater than 50% glomeruli with crescents), referred to the Hospital for Sick Children during the past 13 years, were reviewed. Thirty patients (13 male, 17 female) aged 3.7-15.7 years (mean 9.5) were evaluated. Initial clinical features included: oedema (24/30), hypertension (19/30), gross haematuria (15/30), oliguria (15/30) and a decreased glomerular filtration rate (GFR less than 30 ml/min per 1.73 m2) (22/30). Henoch-Schönlein purpura was present in 9 patients, microscopic polyarteritis in 3, polyarteritis nodosa in 1, Wegener's granulomatosis in 1, systemic lupus erythematosus in 1, post-streptococcal glomerulonephritis in 2, mesangiocapillary glomerulonephritis in 7, anti-glomerular basement membrane glomerulonephritis in 2, and 4 were idiopathic. In 10 patients 50%-79% of glomeruli were affected by crescentic changes (group 1) and in the remaining 20, 80% or more (group 2). The crescents were cellular, fibrocellular or fibrous, and the degree of sclerosis was assessed. Patients in both groups were treated with plasma exchange, corticosteroids, anticoagulants, cyclophosphamide and azathioprine in different combinations. On follow-up, 3 patients were dead, 1 was lost to follow-up, 12 were on dialysis/transplant programmes, 4 had a GFR of less than 30 and 10 a GFR of more than 30 ml/min per 1.73 m2. In our experience, 50% progressed to end-stage renal failure. The interval between disease onset and start of treatment was a prognostic factor for outcome. Fibrous crescents were associated with a worse outcome than fibrocellular crescents (P less than 0.05). Outcome was not, however, related to the percentage of glomeruli affected (P greater than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

  4. Transmembrane TNF-α Reverse Signaling Inhibits Lipopolysaccharide-Induced Proinflammatory Cytokine Formation in Macrophages by Inducing TGF-β: Therapeutic Implications.

    Science.gov (United States)

    Pallai, Anna; Kiss, Beáta; Vereb, György; Armaka, Marietta; Kollias, George; Szekanecz, Zoltán; Szondy, Zsuzsa

    2016-02-01

    TNF-α, a potent proinflammatory cytokine, is generated in a precursor form called transmembrane (m)TNF-α that is expressed as a type II polypeptide on the surface of certain cells. mTNF-α was shown to act both as a ligand by binding to TNF-α receptors, as well as a receptor that transmits outside-to-inside (reverse) signals back into the mTNF-α-bearing cells. In this study, we show that nonactivated macrophages express basal levels of mTNF-α and respond to anti-TNF-α Abs by triggering the MAPK kinase 4 signaling pathway. The pathway induces TGF-β. Based on inhibitory experiments, the production of TGF-β1 is regulated via Jun kinases, whereas that of other TGF-βs is regulated via p38 MAPKs. Exposure to LPS further induced the expression of mTNF-α, and triggering of mTNF-α strongly suppressed the LPS-induced proinflammatory response. Neutralizing TGF-β by Abs prevented the mTNF-α-mediated suppression of LPS-induced proinflammatory cytokine formation, indicating that the immune-suppressive effect of mTNF-α is mediated via TGF-β. Although apoptotic cells are also known to suppress LPS-induced proinflammatory cytokine formation in macrophages by upregulating TGF-β, we show that they do not use the mTNF-α signaling pathway. Because TGF-β possesses a wide range of immune-suppressive effects, our data indicate that upregulation of TGF-β synthesis by those TNF-α-targeting molecules, which are able to trigger mTNF-α, might contribute to their therapeutic effect in the treatment of certain inflammatory diseases such as Crohn's disease, Wegener's granulomatosis, or sarcoidosis. Additionally, none of the TNF-α-targeting molecules is expected to interfere with the immune-silencing effects of apoptotic cells.

  5. Integrated Fluorine-18 Fluorodeoxyglucose (18F-FDG) PET/CT Compared to Standard Contrast-Enhanced CT for Characterization and Staging of Pulmonary Tumors Eligible for Surgical Resection

    Energy Technology Data Exchange (ETDEWEB)

    Quaia, E.; Tona, G.; Gelain, F.; Lubin, E.; Pizzolato, R.; Boscolo, E.; Bussoli, L. (Dept. of Radiology, Cattinara Hospital, Univ. of Trieste, Trieste (Italy))

    2008-11-15

    Background: Accurate staging is necessary to determine the appropriate therapy in patients with lung cancer. Few studies have compared integrated fluorine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) and contrast-enhanced CT in the characterization and staging of pulmonary tumors considered eligible for surgical resection. Purpose: To compare 18F-FDG PET/CT with standard contrast-enhanced CT for the diagnosis and staging of lung neoplasms eligible for surgical resection. Material and Methods: Seventy-six consecutive patients (56 male, 20 female; mean age+-SD, 63.4+-20 years) with 84 pulmonary tumors suspected for malignancy and considered eligible for surgical resection were prospectively enrolled. Seventy-three malignant (65 non-small-cell lung carcinomas, one small-cell lung cancer, two carcinoids, and five metastases) and 11 benign lung tumors (three hamartomas, two sarcoidosis, one amyloidosis, one Wegener granulomatosis, one tuberculosis, and three areas of scarring) were finally diagnosed by histology. Tumor staging was based on the revised American Joint Committee on Cancer. Results: In lesion characterization, the sensitivity and specificity of 18F-FDG PET/CT versus contrast-enhanced CT were 90% vs. 83% and 18% vs. 63% (P<0.05, McNemar test), respectively. In nodal staging, the sensitivity and specificity of 18F-FDG PET/CT versus contrast-enhanced CT were 78% vs. 46% and 80% vs. 93% (P<0.05), respectively. Conclusion: In patients with lung neoplasms considered eligible for surgical resection, 18F-FDG PET/CT versus contrast-enhanced CT revealed higher sensitivity in nodal staging, but lower specificity both in lesion characterization and nodal staging.

  6. Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment.

    Science.gov (United States)

    Duzova, Ali; Bakkaloglu, Aysin

    2008-01-01

    Central nervous system (CNS) manifestations are not rare in pediatric rheumatic diseases. They may be a relatively common feature of the disease, as in systemic lupus erythematosus (SLE) and Behçet's disease. Direct CNS involvement of a systemic rheumatic disease, primary CNS vasculitis, indirect involvement secondary to hypertension, hypoxia and metabolic changes, and drug associated adverse events may all result in CNS involvement. We have reviewed the CNS manifestations of SLE, Behçet's disease, Henoch-Schönlein purpura, polyarteritis nodosa, juvenile idiopathic arthritis, juvenile ankylosing spondylitis, familial Mediterranean fever, scleroderma, sarcoidosis, Wegener's granulomatosis, Takayasu's arteritis, CINCA syndrome, Kawasaki disease, and primary CNS vasculitis; and adverse CNS effects of anti-rheumatic drugs in pediatric patients. The manifestations are diverse; ranging from headache, seizures, chorea, changes in personality, depression, memory and concentration problems, cognitive impairment, cerebrovascular accidents to coma, and death. The value of cerebrospinal fluid (CSF) examination (pleocytosis, high level of protein), auto-antibodies in serum and CSF, electroencephalography, neuroimaging with computerized tomography, magnetic resonance imaging, SPECT, PET, and angiography depends on the disease. Brain biopsy is gold standard for the diagnosis of CNS vasculitis, however it may be inconclusive in 25% of cases. A thorough knowledge of the rheumatic diseases and therapy-related adverse events is mandatory for the management of a patient with rheumatic disease and CNS involvement. Severe CNS involvement is associated with poor prognosis, and high mortality rate. High dose steroid and cyclophosphamide (oral or intravenous) are first choice drugs in the treatment; plasmapheresis, IVIG, thalidomide, and intratechal treatment may be valuable in treatment-resistant, and serious cases.

  7. Meta-Analysis of Associations Between Interleukin-10 Polymorphisms and Susceptibility to Vasculitis.

    Science.gov (United States)

    Jung, Jae Hyun; Song, Gwan Gyu; Lee, Young Ho

    2015-01-01

    This study determined whether interleukin-10 (IL-10) polymorphisms are associated with susceptibility to vasculitis. A meta-analysis was conducted of the associations between the IL-10 -1082 G/A, -819 C/T, and -592 C/A polymorphisms and the haplotype of the IL-10-1082 G/A, -819 C/T, -592 C/A polymorphisms and vasculitis. A total of 21 comparative studies involving 4121 patients and 5504 controls were considered in the meta-analysis. Meta-analysis revealed no association between the IL-10-1082 G allele and vasculitis in all study subjects (OR = 0.927, 95% CI = 0.780-1.102, p = 0.389). However, disease-specific meta-analysis showed an association between Wegener's granulomatosis (WG) and the IL-10-1082 G allele (OR = 0.729, 95% CI = 0.547-0.971, p = 0.031). Meta-analysis revealed an association between vasculitis and the IL-10-819 C allele (OR = 0.804, 95% CI = 0.706-0.916, p = 0.001) in all study subjects and Behcet's disease (BD) (OR = 0.724, 95% CI = 0.679-0.781, p vasculitis in all study subjects (OR = 0.805, 95% CI = 0.619-0.938, p = 0.005) and BD (OR = 0.718, 95% CI = 0.661-0.781, p vasculitis in Europeans (OR = 1.239, 95% CI = 1.105-1.513, p = 0.035). This meta-analysis showed that IL-10 polymorphisms are associated with vasculitis susceptibility, especially in WG and BD.

  8. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century.

    Science.gov (United States)

    Masaki, Yasufumi; Kurose, Nozomu; Umehara, Hisanori

    2011-01-01

    IgG4-related disease is a novel lymphoproliferative disorder that shows hyper-IgG4-γ-globulinemia and IgG4-producing plasma cell expansion in affected organs with fibrotic or sclerotic changes. Patients show systemic inflammatory conditions and various symptoms depending on the affected organ. Since the first report of patients with elevated serum IgG4 in sclerosing pancreatitis in 2001, various systemic disorders described by many names have been reported. Despite similarities in the organs involved in IgG4-related Mikulicz's disease and Sjögren's syndrome, there are marked clinical and pathological differences between these conditions. Most patients diagnosed with autoimmune pancreatitis in Japan have IgG4-related pancreatitis [Type 1 autoimmune pancreatitis (AIP), lymphoplasmacytic sclerosing pancreatitis (LPSP)], a disease distinct from some of the western type [Type 2 AIP, idiopathic duct-centric chronic pancreatitis (IDCP), autoimmune pancreatitis with granulocytic epithelial lesions (GEL)]. Diagnosis of IgG4-related disease is characterized by both elevated serum IgG4 (>135 mg/dL) and histopathological features including lymphocyte and IgG4(+) plasma cell infiltration (IgG4(+) plasma cells/IgG(+) plasma cells>40%). Differential diagnosis from other distinct disorders, such as sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, cancer, and other existing conditions associated with high serum IgG4 level or abundant IgG4-bearing plasma cells in tissues is necessary. We have begun a clinical prospective study to establish a treatment strategy (Phase II prospective treatment study for IgG4-multiorgan lymphoproliferative syndrome: UMIN R000002311).

  9. Clinical significance of routine lacrimal sac biopsy during dacryocystorhinostomy: A comprehensive review of literature.

    Science.gov (United States)

    Koturović, Zoran; Knežević, Miroslav; Rašić, Dejan M

    2017-02-21

    The main purpose of this paper is to provide the information about the incidence and types of pathology of secondary acquired obstructions of the lacrimal excretory outflow system caused by primary lacrimal sac non-neoplastic and neoplastic lesions. After a thorough literature search, 17 case-control studies were found and selected, data were extracted and categorized, to evaluate specific lacrimal sac pathology mimicking inflammation. A total of 3865 histopathologically examined lacrimal sac wall biopsy specimens from 3662 patients, taken during dacryocystorhinostomy for clinically presumed primary chronic dacryocystitis, were analyzed. The most common reported histopathological finding was non-specific chronic inflammation with or without fibrosis (94.15% of cases). Lacrimal sac-specific pathologies were present in 226 (5.85%) cases. Unsuspected lacrimal sac-specific pathologies were present in 55/226 (24.34%) cases. Almost 45% of primary lacrimal sac malignant neoplasms were not suspected, preoperatively and intraoperatively. Tumor-like lesions of the lacrimal sac were the most common pathology found: (1) lacrimal stones-dacryoliths, (2) pyogenic granuloma, (3) granulation tissues, (4) reactive lymphoid hyperplasia, and (5) lacrimal sac-specific inflammation (Wegener's granulomatosis and sarcoidosis). Neoplastic pathology was found in 55/3865 (1.42%) lacrimal sac wall biopsy specimens; of those, malignant cases were 2.24 times more frequent than benign. Lymphoma was the most common preoperatively unsuspected or intraoperatively unexpected neoplastic pathology. This analysis of the relevant literature highlights the value of routine lacrimal sac biopsy during surgery for clinically presumed primary acquired nasolacrimal duct obstruction.

  10. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

    Directory of Open Access Journals (Sweden)

    Fermin Blanco Filho

    2001-01-01

    Full Text Available CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM. Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA, microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14%, platelets = 260,000, white blood cells = 8300, 74% segmented, 4% eosinophils, 19% lymphocytes and 3% monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA test with antigen specificity for myeloperoxidase (anti-MPO was positive and the circulating anti-GBM showed an indeterminate result.

  11. Cutaneous vasculitides: Clinico-pathological correlation

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    Gupta Suruchi

    2009-01-01

    Full Text Available Background: Cutaneous vasculitis presents as a mosaic of clinical and histological findings. Its pathogenic mechanisms and clinical manifestations are varied. Aims: To study the epidemiological spectrum of cutaneous vasculitides as seen in a dermatologic clinic and to determine the clinico-pathological correlation. Methods: A cohort study was conducted on 50 consecutive patients clinically diagnosed as cutaneous vasculitis in the dermatology outdoor; irrespective of age, sex and duration of the disease. Based on the clinical presentation, vasculitis was classified according to modified Gilliam′s classification. All patients were subjected to a baseline workup consisting of complete hemogram, serum-creatinine levels, serum-urea, liver function tests, chest X-ray, urine (routine and microscopic examination besides antistreptolysin O titer, Mantoux test, cryoglobulin levels, antineutrophilic cytoplasmic antibodies and hepatitis B and C. Histopathological examination was done in all patients while immunofluorescence was done in 23 patients. Results: Out of a total of 50 patients diagnosed clinically as cutaneous vasculitis, 41 were classified as leukocytoclastic vasculitis, 2 as Heinoch−Schonlein purpura, 2 as urticarial vasculitis and one each as nodular vasculitis, polyarteritis nodosa and pityriasis lichenoid et varioliforme acuta. Approximately 50% of the patients had a significant drug history, 10% were attributed to infection and 10% had positive collagen workup without any overt manifestations, while 2% each had Wegener granulomatosis and cryoglobulinemia. No cause was found in 26% cases. Histopathology showed features of vasculitis in 42 patients. Only 23 patients could undergo direct immunofluorescence (DIF, out of which 17 (73.9% were positive for vasculitis. Conclusions: Leukocytoclastic vasculitis was the commonest type of vaculitis presenting to the dermatology outpatient department. The workup of patients with cutaneous vasculitis

  12. Endoscopic dacryocystorhinostomy

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    Milojević Milanko

    2010-01-01

    Full Text Available Background/Aim. Intensive epiphora (lacrimal apparatus disease can make difficult daily routine and cause ocular refraction disturbances. In most cases ethiology is unknown, rarely occurs after nose surgical procedures, face fractures, in Wegener granulomatosis, sarcoidosis and malignancies. The aim of this study was to evaluate efficacy of endonasal endoscopic surgical procedure with the conventional surgical instruments in treatment of nasolacrimal obstructions. Methods. This retrospective study included 12 female patients with endonasal endoscopic surgical procedure from Otorhinological and Ophtalmological Departments of Military Medical Academy, Belgrade from September 2007 to April 2009. Preoperative nasal endoscopy was performed in order to reveal concomitant pathological conditions and anatomic anomalies which could make surgical procedure impossible. Computerized tomography was performed only in suspect nose diseases. Surgical endonasal endoscopic procedure was performed by otorhinolaryngologist and ophtalmologist in all patients. The patients had regular controls from 2 to 20 months. Results. A total of 12 female patients, age 34-83 years, were included in our study. Epiphora was a dominant symptom in all patients. In two patients deviation of nasal septum was found, and in other one conha bulosa at the same side as chronic dacryocystitis. All patients were subjected to endonasal dacryocystorhinostomy (DCR by endoscopic surgical technique using conventional instruments. Concomitantly with DCR septoplastics in two patients and lateral lamictetomy in one patient were performed. There were no complications intraoperatively as well as in the immediate postoperative course. In two patients the need for reoperation occurred. Conclusion. Endoscopic DCR is minimally invasive and efficacious procedure for nasolacrymal obstructions performed by otorhinolaryngologist and ophtalmologist. Postoperative recovery is very fast.

  13. Endobronchial resection with the Nd-YAG laser--two years experience in an Australian unit.

    Science.gov (United States)

    Pierce, R J; Mestitz, H; Simpson, L W; Daniel, F J

    1990-04-01

    In a two year period 44 endobronchial resections using the Neodymium-YAG laser have been performed in 28 patients. The majority of cases had either bronchogenic carcinoma (57%) or metastatic carcinoma (18%) involving the bronchial tree. Adenoid cystic carcinoma, benign tumours, lymphoma, tracheal papillomatosis, Wegener's granulomatosis and benign stricture comprised the other cases. Rigid bronchoscopy and general anaesthesia were used in the majority. Symptomatic improvement of dyspnoea when relief of bronchial obstruction occurred was marked in ten of 17 cases, moderate in four and absent in three. Haemoptysis was markedly improved in two of three cases and obstructive pneumonitis resolved in one of two cases. Significant respiratory function improvement was observed in Raw (most sensitive), FEV11, FVC and TLC. Laser treatment restored the lumen to normal calibre in 52% (including all patients with tracheal lesions), to greater than half normal in 28% and to less than half normal in 20% of cases. Re-expansion of a collapsed lung or lobe occurred in seven of eight patients. In six of these patients laser treatment was the initial therapy resulting in immediate re-expansion and symptomatic relief prior to further therapy. In patients with bronchogenic carcinoma the mean time to retreatment or death was 72 days. For metastatic carcinoma this was 60 days. Two early deaths (3 hours, 36 hours) due to respiratory failure occurred in patients with very severe bilateral bronchial obstruction too advanced for effective clearance. Other complications included laryngeal oedema requiring prolonged intubation (1), bronchospasm (1), atrial fibrillation (1), and acute pulmonary oedema (1). Laser treatment provides effective palliation for bronchial obstruction and haemoptysis in selected proximal endobronchial cancers.

  14. Aanwezigheid en gebruik van autogordels en kinderzitjes op de voor- en achterzitplaatsen van personenauto' in 1991 : een onderzoek naar de aanwezigheid en het gebruik van beveiligingsmiddelen door bestuurders en passagiers van personenauto's op wegen binnen en buiten de bebouwde kom.

    NARCIS (Netherlands)

    Verhoef, P.J.G. & Mulder, J.A.G.

    1992-01-01

    During October 1991, a combined survey was done into the presence and use of safety belts and child seats on the back seats of cars. Contrary to previous years, it also looked at the belt use by both drivers and front seat passengers. The use of seat belts by car drivers in 1991 was practically the

  15. Environmentally acceptable mobility in Europe: New strategies for passenger and goods transport. A contribution to ecologically compatible mobility. Documentation; Umweltgerecht mobil in Europa: Menschen und Gueter auf neuen Wegen. Ein Beitrag zur oekologisch vertraeglichen Mobilitaet. Dokumentation

    Energy Technology Data Exchange (ETDEWEB)

    Link, F.G. [Akademie fuer Natur- und Umweltschutz beim Ministerium fuer Umwelt und Verkehr des Landes Baden-Wuerttemberg, Stuttgart (Germany); Diekmann, P.; Holzwarth, J. [comps.] [Ministerium fuer Umwelt und Verkehr des Landes Baden-Wuerttemberg, Stuttgart (Germany)

    1998-11-01

    The traffic sector, with its transport systems and structures which for the most part are not ecologically acceptable, is a significant obstacle to sustainable development. As a contribution to a future-orientated, optimized consideration of both the need for mobility and the necessity of environmental protection, this volume presents big and small steps that can be taken to prevent traffic from the view of politicians, practicians and scientists in the field of passenger and goods transport. (orig.)

  16. Wine, words and action. China's attempts to stop the European antidumping proceedings; Wein, Wort und Gegenwehr. In China wird auf vielen Wegen versucht, das europaeische Antidumping-Verfahren zu stoppen

    Energy Technology Data Exchange (ETDEWEB)

    Bartsch, Bernhard; Eleuteri, Stefano

    2012-11-15

    Chinese manufacturers of solar modules are in fear of losing their biggest market as a consequence of the European antidumping proceedings. In an attempt to protect them, Chinese politicians are now threatening to start a trade war that will go far beyond the solar industry. In spite of the efforts of German chancellor Angela Merkel, the prospects for a compromise are not good.

  17. Rezension von: Laura Adamietz: Geschlecht als Erwartung. Das Geschlechtsdiskriminierungsverbot als Recht gegen Diskriminierung wegen der sexuellen Orientierung und der Geschlechtsidentität. Baden-Baden: Nomos Verlag 2011.

    Directory of Open Access Journals (Sweden)

    Anson Koch-Rein

    2012-01-01

    Full Text Available Laura Adamietz präsentiert in ihrer Dissertation eine präzise Analyse deutschen und internationalen Antidiskriminierungsrechts und bestehender Rechtsprechung in Sachen Geschlecht, sexuelle Orientierung und Geschlechtsidentität. Verbunden wird dies mit einem – nicht nur für ein juristisches Publikum lohnenden – Überblick über einschlägige, aber in der gängigen Rechtsauslegung unzureichend berücksichtigte geschlechterwissenschaftliche Erkenntnisse. Auf dieser Grundlage entwickelt die Autorin eine Auslegung des antidiskriminierungsrechtlichen Begriffs ‚Geschlecht‘ als „Geschlecht als Erwartung“, wodurch dieser gleichzeitig Komplexität und Anwendbarkeit gewinnt. Die Verbindung von theoretischer Sorgfalt, interdisziplinärer Perspektive und Lösungsorientierung ist ein zentrales Verdienst dieses Buches.

  18. In situ, navigational, physical and profile data collected by Alfred Wegener Institute for Polar and Marine Research at OceanSITES site FRAM from 0018-03-19 to 2014-08-31 (NCEI Accession 0139307)

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — In situ, navigational, physical and profile oceanographic data were collected, including CURRENT SPEED - EAST/WEST COMPONENT (U), CURRENT SPEED - NORTH/SOUTH...

  19. Haalbaarheid kencijfers voor lagere-orde-wegen en langzaam verkeer. Deel 2: beschrijving van de aard van de kencijfers. In opdracht van Directoraat-Generaal Rijkswaterstaat, Adviesdienst Verkeer en Vervoer AVV.

    NARCIS (Netherlands)

    Hummel, T.

    1998-01-01

    Since 1988, the SWOV Institute for Road Safety Research has been publishing key risk indexes for road safety that describe the safety level on various road types. In the most recent updating of key risk indexes in 1995, key risk indexes for lower-order roads (residential roads, roads in pedestrian p

  20. Wegdekstroefheid en verkeersongevallen : een onderzoek naar het verband tussen wegdekstroefheid en het relatieve aantal verkeersongevallen op verschillende typen en vormen wegen buiten de bebouwde kom in de provincie Noord-Brabant.

    NARCIS (Netherlands)

    Tromp, J.P.M. Doornekamp, J. & Bos, J.M.J.

    1984-01-01

    A study has been made into the relationship between road surface unevenness and the relative number of traffic accidents on different kinds of roads outside the built-up area in the province of Noord-Brabant. There exists a statistical relation between the road surface unevenness and traffic accide

  1. Discussion of the design of satellite-laser measurement stations in the eastern Mediterranean under the geological aspect. Contribution to the earthquake prediction research by the Wegener Group and to NASA's Crustal Dynamics Project

    Science.gov (United States)

    Paluska, A.; Pavoni, N.

    1983-01-01

    Research conducted for determining the location of stations for measuring crustal dynamics and predicting earthquakes is discussed. Procedural aspects, the extraregional kinematic tendencies, and regional tectonic deformation mechanisms are described.

  2. Recepción temprana de la teoría de la deriva continental y su competencia con las teorías rivales

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    Pérez-Malváez, Carlos

    2003-06-01

    Full Text Available In 1912 Alfred Lothar Wegener proposed the theory of the continental drift. Wegener thought that the displacement explained more paleontological and geophysical data than any other rival program. The great virtue of the drift theory resided in their great capacity of synthesis. Wegener appealed to the information provided by the Geology, Paleontology and Paleoclimatology.

    Alfred Lothar Wegener propuso la teoría de la deriva continental en 1912. Wegener ciertamente pensó que el desplazamiento explicaba mas datos paleontológicos y geofísicos que cualquiera de los programas de investigación establecidos. Desde luego, la gran virtud de la teoría de la deriva fue su gran capacidad de síntesis. Wegener recurrió a la información que provenía de la Geología, Geofísica, Paleontología y Paleoclimatología.

  3. Open lung biopsy

    Science.gov (United States)

    ... CT scan Disseminated tuberculosis Granulomatosis with polyangiitis Lung cancer - small cell Lung disease Lung needle biopsy Malignant mesothelioma Pulmonary tuberculosis Rheumatoid lung disease Sarcoidosis Simple pulmonary eosinophilia ...

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

    Directory of Open Access Journals (Sweden)

    Thais Thomaz Queluz

    2005-07-01

    after the discovery of antineutrophil cytoplasmic antibodies in 1982, and current classifications include Goodpasture's syndrome, forms of vasculitis related to antineutrophil cytoplasmic antibodies, forms caused by immune complexes and other forms. The evidence that antineutrophil cytoplasmic antibodies are involved in the pathogenesis of such conditions led to considerable advances in the diagnosis and treatment of vasculitis. Wegener's granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis, all forms of vasculitis that have been associated with antineutrophil cytoplasmic antibodies, are the systemic forms that most affect the lungs. The most common presentation of such forms is characterized by cough, diffuse alveolar hemorrhage or difficult-to-control asthma. In Takayasu's arteritis, Behçet's disease and Henoch-Schönlein purpura, as well as in forms of vasculitis related to collagen diseases, pulmonary involvement is less common. In all forms of vasculitis, there is evidence that immune system processes are involved. It is based on this knowledge that new therapeutic approaches are proposed.

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

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    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

  6. CLINICAL AND PATHOLOGICAL MANIFESTATI ONS OF PATIENTS WITH ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES DIRECTED AGAINST PROTEINASE 3 OR MYELOPEROXIDASE

    Institute of Scientific and Technical Information of China (English)

    张烜; 董怡; 曾小峰; 李永哲; 唐福林

    2002-01-01

    Objective.To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinase 3 (anti PR3) or myeloperoxidase (anti MPO). Methods.One hundred and forty patients with ANCA were detected for anti PR3 and anti MPO by ELISA.The clinical features at presentation,histopathological characteristics and outcome of all patients who were tested positive for anti PR3 or anti MPO were analysed. Results.In anti PR3 group (n=21),16 cases (76.2% ) had systemic vasculitis,in which Wegener's granulomatosis prevailed (13 cases,61.9% ).In anti MPO group (n=31),19 cases (61.3% ) were diagnosed as systemic vasculitis and 12 cases (38.7% ) as microscopic angiitis.For vasculitic patients with anti PR3 and anti MPO,the disease duration at diagnosis was 9.6± 2.0m and 4.4± 0.9m respectively,P0.05;upper respiratory tract,eye and joint involvements were 11(68.8% ),7(43.8% ),11(68.8% ) and 7(36.8% ),2(10.5% ),5(26.3% ) respectively,P500 μ mol/L were more commonly seen in anti MPO group than in anti PR3 group,which were 8(42.1% ) and 2(12.5% ) respectively,P< 0.05.Ten relapses were seen in anti PR3 group and only 2 in anti MPO group,but the acute mortality rate in anti MPO group (5/19,27.4% ) was much higher than that in anti PR3 group (1/16,6.3% ). Conclusions.Anti PR3 and anti MPO occurred mainly in systemic vasculitis.A large divergence was seen in the disease spectrum between patients with anti PR3 and those with anti MPO.In particular,upper respiratory tract,eye and joint involvements,granuloma formation and relapse were more prominent in anti PR3 patients.By contrast,the anti MPO patients had a more acute disease onset,more rapid progressive renal involvement and a higher acute mortality rate.

  7. High-mobility group box-1 protein (HMGB1) is increased in antineutrophilic cytoplasmatic antibody (ANCA)-associated vasculitis with renal manifestations.

    Science.gov (United States)

    Bruchfeld, Annette; Wendt, Mårten; Bratt, Johan; Qureshi, Abdul R; Chavan, Sangeeta; Tracey, Kevin J; Palmblad, Karin; Gunnarsson, Iva

    2011-01-01

    High-mobility group box 1 (HMGB1) is a nuclear and cytosolic protein that is increasingly recognized as an important proinflammatory mediator actively secreted from monocytes and macrophages and passively released from necrotic cells. In antineutrophilic cytoplasmatic antibody (ANCA)-associated vasculitis (AAV), the kidneys are commonly affected vital organs, characterized by focal necrotizing and/or crescentic pauci-immune glomerulonephritis. The aim of the study was to determine whether HMGB1 serum levels are elevated in AAV with renal manifestations. A total of 30 AAV patients (16 female and 14 male; median age 59 years, range 17-82) with Wegener granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome with available renal biopsies and serum samples were included. In seven cases, serum was also obtained at rebiopsy in remission. HMGB1 was analyzed with Western blot. Birmingham Vasculitis Activity Score (BVAS, version 2003), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), urinanalysis, creatinine, estimated glomerular filtration rate, sex and age were included in the analysis. Twenty-five episodes of biopsy-proven active disease with BVAS 17.9 ± 4.6 and 13 cases with inactive biopsies and BVAS 2.3 ± 3.7 (P = 0.0001) were identified. CRP, ESR, hematuria and proteinuria were significantly higher in active cases. HMGB1 was significantly elevated (P = 0.01) comparing active with inactive cases (120 ± 48 versus 78 ± 46 ng/mL) and significantly lower in the seven control patients (P = 0.03) at rebiopsy in remission. HMGB1 remained higher in inactive cases compared with historic healthy controls (10.9 ± 10.5 ng/mL). HMGB1 levels did not differ significantly between AAV subgroups. CRP and ESR did not correlate with HMGB1. HMGB1 is significantly increased in AAV with renal involvement. Residual HMGB1 elevation in remission could possibly reflect low-grade inflammatory activity or tissue damage. Future studies may further reveal whether HMGB

  8. Clinical spectrum and outcome of crescentic glomerulonephritis in children in developing countries.

    Science.gov (United States)

    Dewan, Deepak; Gulati, Sanjeev; Sharma, Raj K; Prasad, Narayan; Jain, Manoj; Gupta, Amit; Kumar, Alok

    2008-03-01

    Crescentic glomerulonephritis (CsGN) is an uncommon entity in children. This prospective study was conducted to evaluate the aetiology, clinical spectrum and outcome in children with crescentic glomerulonephritis. The single-centre prospective study comprised of 22 children with biopsy proven CsGN who had been referred to our institute over the period January 2000 to December 2005. These patients were subjected to detailed clinical and biochemical examinations. The diagnosis of underlying renal disease was based on various criteria, including the clinical picture, serology and histopathology. The patients received intravenous methyl prednisolone, oral steroid treatment, and oral cyclophosphamide with or without plasmapheresis. All patients received supportive care, including control of hypertension and oedema and supportive management of renal insufficiency. During this 5-year period, CsGN accounted for 5.1% of all biopsies done in children. The mean age was 12.27 years (range 4 years to 18 years). There were eight girls and 14 boys. The mean duration of symptoms prior to referral was 2.47 months (range 5 days to 21 months). Aetiology was immune complex in 19 cases, anti-glomerular basement membrane (anti-GBM) antibody disease in two cases and pauci-immune (Wegener's granulomatosis) in one case. The percentage of crescents ranged from 50% to 100% (mean 70.6%). Twenty-one out of 22 (95.5%) children in our series had hypertension at presentation that required treatment with antihypertensive medications. The serum creatinine level at presentation ranged from 1.5 mg/dl to 11.4 mg/dl (mean 5.5 mg/dl). Of the 22 children, two were lost to follow-up, while the mean follow-up period of the rest of the 20 children was 8.13 months (range 1 month to 43 months). At the last follow-up of the 22 children, ten had stage 5 chronic kidney disease (CKD) and three had stage 4 CKD, while seven children had a calculated glomerular filtration rate (GFR) of >60 ml/min per 1.73 m(2) body

  9. “One-stop shop” spectral imaging for rapid on-site diagnosis of lung cancer: a future concept in nano-oncology

    Directory of Open Access Journals (Sweden)

    Darwiche K

    2013-11-01

    neuroendocrine carcinoma, thymoma, epithelioid and sarcomatoid mesothelioma, cryptogenic organizing pneumonia, malt cell lymphoma, and Wegener's granulomatosis.Conclusion: The CytoViva software, once it had created a specific spectral library for each entity, was able to identify the same disease again in subsequent paired sets of slides of the same disease. Further evaluation of this technique could make this illumination nanotechnology an efficient rapid on-site diagnostic tool.Keywords: lung cancer, endobronchial ultrasound, spectral imaging, diagnosis

  10. Radiologic findings of diffuse Pulmonary hemorrhage

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Mi Ra; Song, Koun Sik; Lee, Jin Seong; Lim, Tae Hwan [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1998-12-01

    To describe the chest radiographic and CT findings of diffuse pulmonary hemorrhage. Two radiologists retrospectively analysed the chest radiographic and CT findings of six patients with diffuse pulmonary hemorrhage. Using open lung biopsy(n=3D2) and transbronchial lung biopsy or bronchoalveolar lavage(n=3D4), diagnosis was based on the presence of hemosiderin-laden macrophage or intra-alveolar hemorrhage. Underlying diseases were Wegener's granulomatosis(n=3D2), antiphospholipid antibody syndrome(n=3D2), Henoch-Schonlein purpura(n=3D1), and idopathic pulmonary hemosiderosis(n=3D1). In all patients, sequential chest radiographs, obtained during a one to six-month period, were available. HRCT scans were obtained in five patinets, and conventional CT scans in one. Follow-up HRCT scans were obtained in two. We also analyzed the patterns of involvement, distribution and sequential changes in the pulmonary abnormalities seen on chest radiographs and CT scans. Chest radiographs showed multifocal patchy consolidation(n=3D6), ground-glass opacity(n=3D3), and multiple granular or nodular opacity(n=3D3). These lesions were intermingled in five patients, while in one there was consolidation only. Sequential chest radiographs demonstrated the improvement of initial pulmonary abnormalities and appearance of new lesions elsewhere within 5-6 days, though within 7-25 (average, 13) days, these had almost normalized. HRCT scans showed patchy consolidation(n=3D5), multiple patchy ground-glass opacity(n=3D5), or ill-defined air space nodules(n=3D4). These lesions were intermingled in five patients, and in one, ground-glass opacity only was noted. In two patients there were interlobular septal thickening and intalobular reticular opacity. The distribution of these abnormalities was almost always bilateral, diffuse with no zonal predominancy, and spared the apex of the lung and subpleural region were less affected. Although chest radiographic and CT findings of diffuse pulmonary

  11. Demonstration of the proliferation marker Ki-67 in renal biopsies: correlation to clinical findings.

    Science.gov (United States)

    Nabokov, A; Waldherr, R; Ritz, E

    1997-07-01

    Assessment of cell proliferation in renal biopsy samples is a potentially promising analytical tool to evaluate disease activity. So far no information is available on the correlation between proliferative activity in different anatomic compartments of the kidney and clinical symptoms. To elucidate this issue, we examined renal biopsy specimens from 20 patients with systemic vasculitis (15 Wegener's granulomatosis, five microscopic polyangiitis), 20 patients with immunoglobulin (Ig) A nephropathy (IgAN), 13 patients with minimal-change disease (MCD), 11 patients with tubulointerstitial nephritis, and five patients with diabetes mellitus. The streptavidin-biotin-peroxidase complex technique was applied to autoclave-pretreated, formalin-fixed, paraffin-embedded tissue sections to label different cell types with the antibody MIB1 directed against the Ki-67 antigen. Proliferation index (PI) was estimated as the number of positively stained nuclei per glomerular cross-section or per square millimeter section area. The interstitial cells were discriminated by additional staining of Ki-67-processed samples with specific immune markers. In patients with vasculitis, PI was considerably elevated in the extracapillary glomerular compartment (0.86), in proximal tubules (6.24), and in the interstitium (8.62). High proliferative activity was also noted in interstitium (3.98) and proximal tubules (1.35) of patients with IgAN. Of particular interest was the increased interstitial proliferative activity (15.0) in diabetic patients. Resident renal cells, but not infiltrating cells, seemed to constitute the majority of the proliferating cell population in the interstitium. In systemic vasculitis, clinical disease activity was significantly correlated to endocapillary (r(s) = 0.58), extracapillary (r(s) = 0.67), proximal tubular (r(s) = 0.67), and interstitial PI (r(s) = 0.61). By multiple linear regression analysis, proximal tubular PI was correlated to the presence of hematuria

  12. Hemorragia alveolar maciça como manifestação inicial de poliangeíte microscópica Diffuse alveolar hemorrhage as initial manifestation of microscopic polyangiitis

    Directory of Open Access Journals (Sweden)

    Cristiano Barbosa Campanholo

    2007-02-01

    Full Text Available Hemorragia alveolar (HA é uma manifestação clínica com alta taxa de mortalidade que deve ser investigada, reconhecida e estabilizada. Causas possíveis para a HA incluem infecções respiratórias ou sistêmicas, malformações arteriovenosas, estenose mitral, discrasias sangüíneas e doenças auto-imunes, como o lúpus eritematoso sistêmico (LES, a síndrome de Goodpasture e as vasculites sistêmicas primárias, principalmente aquelas associadas aos anticorpos anticitoplasma de neutrófilos (Anca, como a granulomatose de Wegener, síndrome de Churg-Strauss e a poliangeíte microscópica. Relatamos o caso de uma paciente jovem que apresentou quadro grave de HA necessitando ventilação mecânica assistida com pressão expiratória final positiva (Peep. Na ausência de evidências de infecção, discrasias sangüíneas ou malformações arteriovenosas, a paciente foi submetida à corticoterapia e à imunossupressão, com controle satisfatório da atividade da doença, que persiste após 24 meses de seguimento. Os dados clínicos, laboratoriais e histopatológicos permitiram estabelecer o diagnóstico de poliangeíte microscópica.Alveolar hemorrhage (AH is a clinical manifestation with high mortality rate that must be promptly investigated, recognized and stabilized. Causes of AH include systemic and respiratory tract infections, arterio-venous malformations, blood dyscrasias and autoimmune diseases such as systemic lupus erithematosus, Goodpasture syndrome and primary systemic vasculitis, specially the antineutrophil cytoplasmic antibodies-associated vasculitis such as Wegener’s granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis. We report the case of a young female patient who developed severe AH necessitating orotracheal intubation and mechanic assisted ventilation with positive end expiratory pressure. Since no evidence of infection, malformation or blood dyscrasia was found, immediate therapy with glucocorticoids and

  13. Recommendations for speed management strategies and policies

    NARCIS (Netherlands)

    Kallberg, V.P.; Allsop, R.; Ward, H.; Horst, A.R.A. van der; Varhelyi, A.

    1998-01-01

    Aanbevelingen worden gedaan voor harmonisatie van snelheden op 4 typen wegen in Europa en voor middelen om verkeersveilige snelheden te bewerkstelligen (waaronder intelligente snelheidsadaptatie in de auto).

  14. Kontinental-Verschiebungen : Originalnotizen und Literaturauszüge = Continental Drift : the Original Notes and Quotations

    OpenAIRE

    2005-01-01

    Alfred Wegeners Notizbuch "Kontinental-Verschiebungen" : eine Einführung / von Reinhard Krause und Jörn Thiede: 2005 jährt sich der Geburtstag Alfred-Wegeners zum 125. Mal (geboren 1. November 1880). Bei den Vorbereitungen auf dieses Jubiläum und auf die Feier des 25- jährigen Bestehens der Stiftung Alfred-Wegener-Institut für Polar- und Meeresforschung sind die Unterlagen, die auf Alfred Wegener persönlich zurückgehen, sorgfältig durchgesehen worden. Bei seinem Enkel Dr. Günther Sc...

  15. 嗜酸性肉芽肿性多血管炎合并坏疽一例并文献复习%Eosinophilic granulomatosis with polyangiitis complicated with gangrene:one case report and literature ;review

    Institute of Scientific and Technical Information of China (English)

    莫颖倩; 林建子; 李红; 郑东辉; 戴冽

    2016-01-01

    目的:探讨嗜酸性肉芽肿性多血管炎(EGPA)合并坏疽的诊治要点。方法报道1例EGPA合并指端坏疽患者,以“EGPA”或“Churg-Strauss综合征”或“变应性肉芽肿(性)血管炎”以及“坏疽”或“缺血”为检索词,对以下数据库的相关论文进行检索:生物医学文献数据库(MEDLINE)、美国国家生物技术信息中心(NCBI)、中国生物医学文献数据库(CBM)、中国期刊全文数据库(CNKI)及万方数据知识服务平台等,筛选出EGPA合并坏疽患者的临床资料进行总结。结果该例为48岁男性患者,于2年内出现反复发热、皮疹、游走性肺浸润伴肺出血、上颌窦黏膜下囊肿、下肢深静脉血栓形成、右手食指指端坏疽、右手臂闪电样疼痛,外周血嗜酸性粒细胞显著升高、血小板减少,诊断为EGPA合并指端坏疽,继发性抗磷脂抗体综合征(APS),给予大剂量激素加环磷酰胺以及抗凝等治疗,治疗7周后右手食指指尖破溃处基本愈合,其余表现均好转。检索文献,收集4例有详细临床资料的确诊为EGPA合并坏疽的国外患者,其中2例为单发指端坏疽、1例为多发指端坏疽、1例为足坏疽,2例接受激素、免疫抑制剂、抗凝等治疗后好转,1例行指端截肢手术后好转,1例因足坏疽进展而死亡。结论 EGPA合并坏疽较罕见,诊断时需注意排除遗传性易栓症及APS等获得性易栓症。EGPA合并坏疽需及早治疗,否则预后欠佳。%Objective Todiscussthediagnosisandtreatmentofeosinophilicgranulomatosiswith polyangiitis(EGPA)complicatedwithgangrene.Methods OnemalepatientofEGPAcomplicatedwithgan-grene was reported. Relevant literatures were searched in the MEDLINE,NCBI,CBM,CNKI,and WAN-FANG databases using the keywords of“EGPA”or “Churg-Strauss syndrome”or “allergic granulomatosis”combined with“gangrene”or“ischemia”. Clinical data of EGPA patients complicated with gangrene were re-viewed.Results Themalepatient,aged48years,presentedwithprolongedfever,rash,wanderingpulmona-ry infiltration complicated with pulmonary hemorrhage,submucosal cyst in maxillary sinus,deep venous throm-bosis in lower extremities,gangrene in the right index finger of right hand,lightning-like pain in the right arm, eosinophilia and thrombocytopenia in the recent 2 years. He was diagnosed with EGPA combined with gangrene and secondary antiphospholipid syndrome (APS). After 7-week therapy with high-dose glucocorticoid,cyclo-phosphamide and anticoagulant,gangrene in the index finger of right hand was recovered and other clinical manifestations were improved. According to literature review,4 cases with details of EGPA complicated with gangrene were reported aboard,2 had gangrene in single fingertip,1 had gangrene in multiple fingertips and 1 had foot gangrene. Two patients were successfully treated with glucocorticoid,immunosuppressant and anticoag-ulant,one was recovered after undergoing amputation surgery and another patient died from aggravated foot gan-grene.Conclusions EGPAcomplicatedwithgangreneisrarelyencounteredinclinicalsettings.Thepossibili-ty of inherited thrombophilia,APS and acquired thrombophilia should be excluded when the diagnosis is made.Due to poor prognosis for EGPA combined with gangrene,early intervention is urgently required.

  16. UNUSUAL TYPE OF NODULAR LUNG DISEASE IN RHEUMATOID ARTHRITIS: A RARE CASE

    Directory of Open Access Journals (Sweden)

    Irina V Liskina

    2014-01-01

    Full Text Available The article presents a rare case of pulmonary manifestation of seropositive protracted rheumatoid arthritis in the form of bronchocentric granulomatosis. The algorithm for diagnosis is described in details in the context of the known literature on pulmonary manifestations as a complication of rheumatoid arthritis and morphology of bronchocentric granulomatosis.

  17. Zur Geschichte der Geophysik

    Science.gov (United States)

    Strobach, Klaus

    1980-07-01

    Alfred Wegener's most important work, the theory of continental drift, has a key position in the history of geophysics and has crucially advanced the discussion of this central problem of geodynamics amongst supporters and adversaries. The aim of this paper is to paint a portrait of Wegener's personality, of his stations of life, and of his interests and research work. The conceptions of the origin of continents and oceans prior to Wegener, and the further development of his ideas after his death on the ice cup of Greenland 50 years ago are discussed.

  18. Didaktiker des Fremdsprachenunterrichts auf neuen Wegen. (Burgschmidt, E.; Goetz, D.; Hoffmann, H. G.; Hohmann, H.-O.; Schrand, H.: "Englisch als Zielsprache. Handbuch des Englischunterrichts unter besonderer Beruechsichtigung der Weiterbildung." Muenchen, Hueber, 1975) (Trainers of Foreign Language Teachers on New Paths)

    Science.gov (United States)

    Hartig, Paul

    1975-01-01

    This article reviews the handbook's five chapters on: 1) psycholinguistics and psychology of learning, 2) questions of content, 3) hints on methodology, with examples, 4) teaching aids, 5) chicking on achievement. All chapters stress continuing education (adult level), and offer valuable, practical suggestions. (Text is in German.) (IFS/WGA)

  19. Aanwezigheid en gebruik van autogordels op de voorzitplaatsen van personenauto's in 1990 : twaalf jaar IMA-methoden bij onderzoek naar het gebruik van autogordels : verslag van waarnemingen gedaan bij bestuurders en voorpassagiers van personenauto's op wegen binnen en buiten de bebouwde kom.

    NARCIS (Netherlands)

    Verhoef, P.J.G.

    1991-01-01

    The results of a surveillance method used in 1990 to ascertain the development of seat belt usage in the Netherlands are described in this report. The usage in outside built-up areas was in 1990 the same as in 1989, namely 78%. The increase in seat belt use since 1986 seems to have definitively come

  20. Listeriosis

    Science.gov (United States)

    Listerial infection; Granulomatosis infantisepticum; Fetal listeriosis ... In infants, symptoms of listeriosis may be seen in the first few days of life and may include: Loss of appetite Lethargy Jaundice Respiratory distress ( ...

  1. [Neurologic and psychiatric syndromes of rare para-rheumatic diseases (author's transl)].

    Science.gov (United States)

    Gottwald, W

    1977-03-01

    In Wegener's granulomatosis peripheral nerves are more often involved than central nervous structures; functional psychoses are secondary e.g. to renal complications. Neurologic-psychiatric symptoms are varied and result from local spread from the upper respiratory tract with separate granulomata and vasculitis. Striped muscle is involved in about 10% of cases. Specific vegetative disturbances of function do not appear to occur. This is also true for Granuloma gangrenescens. Its multicentric inflammatory, ulcerating and necrotic foci involve midline structures and often start at the anterior neuroporus. Becet's disease, however, shows clear characteristic localisation, symmetry of various dermatologic phenomena in the extremities and the almost obligatory involvement of mouth, stomach and the genitals and their surroundings. This one may assume to be due to a mesencephalic parasympathicotonic reaction as the basis for the occurrence of perioral and acro-syndroms after Fischer-Brügge and Sunder-plassmann. Some similarity with the localization of other collagenous diseases is evident. The anterior basal ganglia represent a favored focus where e.g. initial, imcomplete and readily recurrent processes, possibly of an exsudative nature, may start off neurovascular changes of limited areas without necessarily producing clinically detectable signs of the usual neurologic-psychiatric type. In Behcet's disease, rather as in scleroderma, the nervous system appears to be decisively involved. About one third of the cases show changes in the nervous system ("Neuro-Behcet" in the usual sense) with little peripheral signs and rare myositis. But Behcet's disease may also-like erythematodes or progressive sclerodermia-simulate a disseminated encephalomyelitis. The pathogenetic factors of Reiter's syndrome appear to be closely related. The relatively constant main symptoms are also localized and permit the conclusion that they depend on vasomotorically linked innervation of the

  2. Risk of subsequent ischemic and hemorrhagic stroke in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden

    Directory of Open Access Journals (Sweden)

    Zöller Bengt

    2012-06-01

    Full Text Available Abstract Background Certain immune-mediated diseases (IMDs have been associated with increased risk for cardiovascular disorders. The aim of the present study was to examine whether there is an association between 32 different IMDs and first hospitalization for ischemic or hemorrhagic stroke. Methods All individuals in Sweden hospitalized with a main diagnosis of IMD (without previous or coexisting stroke, between January 1, 1987 and December 31, 2008 (n = 216,291, were followed for first hospitalization for ischemic or hemorrhagic stroke. The reference population was the total population of Sweden. Adjusted standardized incidence ratios (SIRs for ischemic and hemorrhagic stroke were calculated. Results Totally 20 and 15 of the 32 IMDs studied, respectively, were associated with an increased risk of ischemic and hemorrhagic stroke during the follow-up. The overall risks of ischemic and hemorrhagic stroke during the first year after hospitalization for IMD were 2.02 (95% CI 1.90–2.14 and 2.65 (95% CI 2.27–3.08, respectively. The overall risk of ischemic or hemorrhagic stroke decreased over time, to 1.50 (95% CI 1.46–1.55 and 1.83 (95% CI 1.69–1.98, respectively, after 1–5 years, and 1.29 (95% CI 1.23–1.35 and 1.47 (95% CI 1.31–1.65, respectively, after 10+ years. The risk of hemorrhagic stroke was ≥2 during the first year after hospitalization for seven IMDs: ankylosing spondylitis (SIR = 8.11, immune thrombocytopenic purpura (SIR = 8.60, polymyalgia rheumatica (SIR = 2.06, psoriasis (SIR = 2.88, rheumatoid arthritis (SIR = 3.27, systemic lupus erythematosus (SIR = 8.65, and Wegener´s granulomatosis (SIR = 5.83. The risk of ischemic stroke was ≥2 during the first year after hospitalization for twelve IMDs: Addison’s disease (SIR = 2.71, Crohn´s disease (SIR = 2.15, Grave´s disease (SIR = 2.15, Hashimoto´s thyroiditis (SIR = 2.99, immune thrombocytopenic purpura (SIR = 2

  3. Clinical retrospective analysis of hemoptysis in 104 children%咯血患儿104例临床回顾分析

    Institute of Scientific and Technical Information of China (English)

    马渝燕; 焦安夏; 饶小春; 潘跃娜; 刘玺诚

    2012-01-01

    Objective To analyze the clinical characters , etiology constituent and bronchoscopy manifestation of children with hemoptysis. Methods The study was performed in 104 children diagnosed with hemoptysis during 2002.1-2011.3 in Beijing Children's Hospital. According to their clinical characters, radiologic manifestation and bronchoscopy outcome, sum up the etiology constituent of children with hemoptysis. Results In 104 cases, male: female=49:55. Id-iopathic pulmonary hetnosiderosis (IPH) 24 cases,bronchiolitis 29 cases,pneumonia 11 cases,bronchiectasis 7 cases, endobronchial tuberculosis 3 cases, foreign body in bronchus 5 cases, bronchial arterial and pulmonary arterial fistula 6 cases, pulmonary vein obstruction or agenesis 3 cases, suspicious of bronchial pulmonary vessel dysplasia 10 cases (requiring angiogram) .congenital cystic adenomatoid malformation(CCAM) of lung 1 case, bronchial inflammatory pseudo-tumor lease, tumor of bronchus 1 case, Wegener granulomatosis 1 case, undifferentiated connective tissue disease 1 case,resistant ANCA small vessel vasculitis lease. Conclusion In conclusion, acute or chronic low airway infections, idiopathic pulmonary hemosiderosis (IPH) and congenital branch pulmonary vessels dysplasia are the first three etiology of hemoptysis in children.%目的 分析儿童咯血病因构成、临床特点及支气管镜表现.方法 收集2002年1月至2011年3月于北京儿童医院住院治疗的104例咯血患儿临床资料,分析其临床特点、影像学特点和支气管镜表现,总结儿童咯血的病因构成.结果 104例中特发性肺含铁血黄素沉着症24例、支气管炎29例、肺炎11例、支气管扩张症7例、支气管内膜结核3例、支气管异物5例、支气管动脉肺动脉瘘6例、肺静脉闭塞或缺如3例、疑诊支气管-肺血管发育异常10例(未行血管造影确诊)、肺囊性腺瘤样畸形1例、支气管黏液表皮样癌1例、支气管炎性假瘤1例、韦格纳肉芽肿1

  4. Case note: EHRM (zaaknummer 36769/08: Ashby Donald ea/Frankrijk)

    NARCIS (Netherlands)

    Hugenholtz, P.B.

    2015-01-01

    Geaccrediteerde fotografen maken beeldmateriaal van Parijse modeshows beschikbaar op door hen beheerde website. Zij worden wegens auteursrechtinbreuk veroordeeld tot strafrechtelijke boetes en schadevergoeding aan modehuizen en hun beroepsvereniging. Verzoekers beroepen zich op hun vrijheid van meni

  5. Geluidbelasting in het centraal Veluws natuurgebied; een quick scan van de geluidbelasting in het centraal Veluws natuurgebied in zijn geheel en in afzonderlijke delen die belangrijk zijn voor recreatie

    NARCIS (Netherlands)

    Goossen, C.M.; Langers, F.

    2003-01-01

    Dit onderzoek geeft inzicht in de knelpunten tussen recreatie en geluidbelasting in het centraal Veluws natuurgebied. Via een GIS-analyse zijn de geluidbelastingbestanden van snelwegen, provinciale wegen, spoorlijnen en luchtvaart geconfronteerd met het centraal Veluws natuurgebied, een bestand met

  6. Windturbines op veilige afstand

    NARCIS (Netherlands)

    Riedstra, D

    2005-01-01

    Initiatieven voor windmolenparken stuiten vaak op verzet wegens horizonvervuiling en vogelsterfte. Veiligheidsrisico’s worden slechts weinig als bezwaar aangevoerd. Toch zijn windturbines niet geheel ongevaarlijk. De aanwezigheid van gevaarlijke stoffen in de nabije omgeving is wel degelijk een fact

  7. Speed-reducing measures for 80 km/h roads

    NARCIS (Netherlands)

    Horst, A.R.A. van der

    1997-01-01

    Op 80-km wegen lijkt de snelheid alleenverlaagd te kunnen worden metfysieke maatregelen aan de weg. In Drenthe is een basispakket ontwikkeld, mede op basis van een simulatorstudie en in de praktijk getest met een succesvol resultaat.

  8. Depth-First Search and Strong Connectivity in Coq

    OpenAIRE

    Pottier, François

    2015-01-01

    National audience; Using Coq, we mechanize Wegener's proof of Kosaraju's linear-time algorithm for computing the strongly connected components of a directed graph. Furthermore, also in Coq, we define an executable and terminating depth-first search algorithm.

  9. Defragmentation measures and the increase of a local European badger (Meles meles) population at Eindegooi, the Netherlands

    NARCIS (Netherlands)

    Vink, J.; Apeldoorn, van R.C.; Bekker, G.J.

    2008-01-01

    Ontsnipperingsmaatregelen en de groei van de lokale dassenpopulatie (Meles meles) op Eindegooi . Vanaf 1984 zijn gegevens verzameld op het landgoed Eindegoed en omgeving. De das koloniseerde nieuwe terreinen. In dezelfde periode zijn vele ontsnipperende maatregelen bij wegen getroffen.

  10. Toolmanagement - Werkzeuge und Prozesse als Schlüssel für eine effiziente Produktion

    Science.gov (United States)

    Enßle, Magnus

    In Bezug auf die Fertigungskosten nehmen die Werkzeugkosten mit 3% einen sehr kleinen und daher vermeintlich unwichtigen Posten ein. HoheKosten ergeben sich aber meist indirekt durch Maschinenstillstandszeiten wegen fehlender oder falscher Werkzeuge, wegen ineffektiven Rüstvorgängen, umständlicher Werkzeuglogistik, unnötig hoher Bestände, unabgestimmter Abläufe, nicht aktueller Fertigungsunterlagen sowie mangelhafter Informationsflüsse zwischen Planung, Vorbereitung und Ausführung in der Fertigung [1].

  11. Development of a Facility for Combustion Stability Experiments at Supercritical Pressure

    Science.gov (United States)

    2013-12-01

    Supercritical Pressure 5a. CONTRACT NUMBER In-House 5b. GRANT NUMBER 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Wegener, Leyva, Forliti, Talley 5d... supercritical pressures that are relevant to high-performance liquid rocket engines, accurately-controlled and cryogenically-conditioned propellants, and... Supercritical Pressure Jeffrey L. Wegener1 University of California at Los Angeles, CA 90095 Ivett A. Leyva2 AFRL/RQRE, Edwards AFB, CA 93524

  12. Precambrian crustal evolution in relation to geodynamics and supercontinents: Preface

    Science.gov (United States)

    Abu-Alam, Tamer S.; Santosh, M.; Tsunogae, Toshiaki

    2016-10-01

    More than a century ago, Alfred Wegener explored the origin of continents in his work: "Die Entstehung der Kontinente" (Wegener, 1912, 1915) which provided the core idea of plate tectonics. However, significantly before this work, the idea of "continental drift" was hypothesized by Abraham Ortelius in 1596 with the suggestion that the Americas were torn away from Europe and Africa by earthquakes and floods, and that these continents were once united.

  13. Manifestações destrutivas da córnea e esclera associadas a doenças do tecido conectivo: relato de 9 casos Corneal and scleral destructive involvement associated with connective tissue disease: report of 9 cases

    Directory of Open Access Journals (Sweden)

    Namir Clementino Santos

    2004-08-01

    apropriado da doença de base.PURPOSE: To study the clinical features, diagnosis, treatment, and outcome of nine patients with corneal and scleral involvement associated to connective tissue disease. METHODS: Description of anterior segment abnormalities observed in nine patients with connective tissue disease, five of them previously diagnosed and four with the ocular involvement being the first manifestation of the disease. All patients were evaluated at the Corneal and External Diseases outpatient clinic of the Federal University of São Paulo - Escola Paulista de Medicina (UNIFESP-EPM between July/1999 to December/2000 and were submitted to a complete ophthalmologic examination, clinical evaluation and laboratory investigation. Clinical or surgical treatment was indicated according to the ocular disease evolution. RESULTS: The clinical diagnoses of the patients with anterior segment inflammatory disease were rheumatoid arthritis in seven patients (77.8%, systemic sclerosis and Wegener´s granulomatosis in each of the remaining patients (22.2%. The most frequent ocular manifestation was scleritis (66.6%, followed by peripheral ulcerative keratitis (55.5% and dry eye syndrome (44.4%. Eighty-nine percent (89% of the patients required immunosuppressive therapy to control the ocular inflammatory disease. Remission of the ocular symptoms were observed after 3 months of the beginning of imunossupressive therapy on average. In 55.5% of the patients a surgical approach (conjunctival resection, application of cyanoacrylate tissue adhesive, penetrating keratoplasty and scleral patch graft was necessary. CONCLUSION: The finding of corneal and scleral involvement associated with connective tissue disease is a sign of the disease activity and usually requires imunossupressive therapy. The ophthalmologists should be aware of these conditions in order to early diagnose and apply the appropriate treatment.

  14. Anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis: two case reports and review of literature

    Directory of Open Access Journals (Sweden)

    Spoerl David

    2012-12-01

    Full Text Available Abstract Background Anti-neutrophil cytoplasmic antibodies are typically detected in anti-neutrophil cytoplasmic antibody associated vasculitis, but are also present in a number of chronic inflammatory non-vasculitic conditions like rheumatoid arthritis. Rare cases of granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis, a vasculitic disorder frequently associated with the presence of anti-neutrophil cytoplasmic antibodies in patients with rheumatoid arthritis have been described in literature. Case presentation We report two middle-aged female patients with rheumatoid arthritis who developed anti-neutrophil cytoplasmic antibodies and symptoms reminiscent of granulomatosis with polyangiitis. Despite the lack of antibodies specific for proteinase 3 and the absence of a classical histology, we report a probable case of granulomatosis with polyangiitis in the first patient, and consider rheumatoid vasculitis in the second patient. Conclusion Taken together with previous reports, these cases highlight that anti-neutrophil cytoplasmic antibodies have to be evaluated very carefully in patients with rheumatoid arthritis. In this context, anti-neutrophil cytoplasmic antibodies detected by indirect immunofluorescence appear to have a low diagnostic value for granulomatosis with polyangiitis. Instead they may have prognostic value for assessing the course of rheumatoid arthritis.

  15. Vasculitis for the internist: focus on ANCA-associated vasculitis.

    Science.gov (United States)

    Chaigne, Benjamin; Guillevin, Loïc

    2017-08-01

    Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality. Herein, after reviewing the literature, we concentrate on selected aspects important for the internist, including classification, diagnostic dilemmas, treatment novelties, and follow-up.

  16. Intralymphatic granulomas as a pathogenic factor in cheilitis granulomatosa/Melkersson-Rosenthal syndrome: report of a case with immunohistochemical and molecular studies.

    Science.gov (United States)

    González-García, Carmen; Aguayo-Leiva, Ingrid; Pian, H; Fernández-Guarino, Montserrat; Jaén-Olasolo, Pedro

    2011-08-01

    Orofacial granulomatosis, an uncommon immunologically mediated disorder, includes cheilitis granulomatosa and Melkersson-Rosenthal syndrome. It is clinically characterized by recurrent or persistent swelling of the orofacial tissues with a spectrum of other orofacial features and sometimes with neurological symptoms. The pathological findings are varied but are often characterized by the presence of noncaseating granuloma. We present a new case of orofacial granulomatosis with unusual histopathological findings, namely, intralymphatic granulomas. These may be the cause of the tissue edema. We demonstrated, by immunohistochemical studies, the lymphatic nature of the vessels affected by the granulomatous process.

  17. Solitary pulmonary nodule

    Science.gov (United States)

    ... Chest x-ray Granulomatosis with polyangiitis Histoplasmosis Lung cancer - small cell Pulmonary tuberculosis Skin nodules Valley fever Review Date 8/1/2015 Updated by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, ... Cancer Lung Diseases Browse the Encyclopedia A.D.A. ...

  18. Chemokine receptor co-expression reveals aberrantly distributed T-H effector memory cells in GPA patients

    NARCIS (Netherlands)

    Lintermans, Lucas L.; Rutgers, Abraham; Stegeman, Coen A.; Heeringa, Peter; Abdulahad, Wayel H.

    2017-01-01

    Background: Persistent expansion of circulating CD4(+) effector memory T cells (TEM) in patients with granulomatosis with polyangiitis (GPA) suggests their fundamental role in disease pathogenesis. Recent studies have shown that distinct functional CD4(+) TEM cell subsets can be identified based on

  19. Think twice – Diagnostic delay in a patient with acute chest pain

    DEFF Research Database (Denmark)

    Bang, Caecilie Larsen; Porsbjerg, Celeste

    2016-01-01

    Heart involvement is the most critical and potentially lethal systemic manifestation in eosinophilic granulomatosis with polyangiitis (EGPA). We present a case of acute chest pain in a 58-year-old male with severe asthma, which regressed after sublingual administration of nitroglycerine. At the t...

  20. [Mediastino-pulmonary sarcoidosis].

    Science.gov (United States)

    Delaval, P; Desrues, B; Quinquenel, M L; Lineau, C; Lena, H

    1993-01-01

    Sarcoidosis is diffuse granulomatosis disease. The aetiology and pathogenesis are unknown. Many different localizations have been described together with immunological disturbances. Generally, the prognosis is favourable. Here we describe the pulmonary manifestations and their staging. Extra-pulmonary manifestations are then presented. Finally, the principle elements of the diagnosis and the treatment of sarcoidosis are discussed.

  1. Detection of antineutrophil cytoplasmic antibodies (ANCAs)

    DEFF Research Database (Denmark)

    Damoiseaux, Jan; Csernok, Elena; Rasmussen, Niels

    2017-01-01

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

  2. Somatostatin analogue scintigraphy and tuberculosis: case report; Scintigraphie des analogues de la somatostatine et tuberculose: a propos d'un cas

    Energy Technology Data Exchange (ETDEWEB)

    Biancheri, I.; Rudenko, B.; Vautrin, P.; Raddoul, J.; Lamfichek, N. [Centre Hospitalier General Andre Boulloche, 25 - Montbeliard (France); Kantelip, B.; Mantion, G. [Centre Hospitalier Universitaire Jean Minjoz, 25 - Besancon (France)

    2005-05-15

    Scintigraphy using a radiolabelled somatostatin analogue (111 In-pentetreotide) is useful in the detection of neuroendocrine tumors. But this radiopharmaceutical accumulates also in solid tumours or in inflammatory diseases such as granulomatosis. We present a case of 111 In-pentetreotide uptake in a tuberculous adenopathy. (author)

  3. AÑO 2011. VOLUMEN 2

    Directory of Open Access Journals (Sweden)

    various

    2011-12-01

    Full Text Available Nº1- SÍNDROME DE WILLIAMS-BEUREN. ESTUDIO DE UN CASOMª Elvira Santos Pérez1-14Nº2-LINFOEPITELIOMA PAROTIDEO. UN HALLAZGO INFRECUENTE.Pilar Pomar Blanco, Nuria Rodríguez Prado, Laura Diez González, Fernando Campo Cerecedo, Jesús San Román Carbajo, Cristina Martín Villares, Manuel Tapia Risueño.15-28Nº3- COMUNICACIONES LIBRES DEL XIX CONGRESO. SANTILLANA DEL MAR (CANTABRIA, 3 Y 4 DE JUNIO DE 2011Varios autores29-63Nº4- MEMORIA ANUAL 2011. REVISTA DE LA SOCIEDAD OTORRINOLARINGOLÓGICA DE CASTILLA Y LEÓN, CANTABRIA Y LA RIOJAJosé Luis Pardal-Refoyo64-82Nº5- TROMBOSIS DEL SENO LATERAL. COMPLICACIÓN DE UNA OTITIS MEDIA SUBAGUDARebeca de la Fuente Cañibano, Santiago Santa Cruz Ruiz83-91Nº6- DISFUNCIÓN DE LAS CUERDAS VOCALES EN LA INFANCIA Y ADOLESCENCIA. A PROPÓSITO DE UN CASONavazo Eguía AI; Arias Tobalina H; Suárez-Muñiz E; De La Mata Franco G92-99Nº7- GRANULOMATOSIS DE WEGENER. MANIFESTACIONES OTORRINOLARINGOLÓGICAS. A PROPÓSITO DE UN CASONavazo-Eguía AI; Grijalba-Uche M; Rivas-Salas A; Suárez-Muñiz E; de-Llano-Varela P100-107Nº8- SÍNDROME DE PFEIFFER. A PROPÓSITO DE UN CASONavazo- Eguía AI, Suárez-Muñiz E, García-Vicario F, de la Mata- Franco G, Suárez-Fernández J, Gómez-Sáez F108-115Nº9- ALTERACIONES DEL LENGUAJE EN PACIENTES AFECTOS DE SÍNDROME DE DOWNMª Elvira Santos Pérez, Cristina Bajo Santos116-134Nº10- IMAGEN: ABSCESO RETROFARÍNGEO TUBERCULOSOPedro Díaz de Cerio Canduela135-139Nº11- LINFANGIOMA AMIGDALINO POLIPOIDEEnrique Coscarón Blanco ,Eva Purificación Martín Garrido, Inmaculada Ursúa Sarmiento140-150Nº12- QUISTES NO FUNCIONANTES DE PARATIROIDES: DIAGNÓSTICO Y TRATAMIENTOJosé Luis Pardal Refoyo; Inmaculada Ursúa Sarmiento151-174Nº13- LENGUA PLICATA: DEBUT DEL SÍNDROME DE MELKERSSON-ROSENTHALCrespo Escudero P; Ibáñez Muñoz C; Tejero Cavero M; Salcedo de la Cruz W; Escobar Martínez M175-178Nº14- ABSCESO RETROFARINGEO COMO COMPLICACIÓN DE MONONUCLEOSIS INFECCIOSAAmaya Rold

  4. IgG4在鼻腔鼻窦炎性病变组织中的表达及其意义%Expression and significance of IgG4 in inflammatory disease of nasal cavity and paranasal sinuses

    Institute of Scientific and Technical Information of China (English)

    吕晶; 刘红刚

    2013-01-01

    cases,mild (10 to 29 per high-power field) in 30 cases and negative (< 10 per high-power field) in 22 cases (P <0.05).Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (> 50 IgG4-positive plasmacells per high-power field and IgG4-to-IgG ratio > 40%),including 3 cases of chronic sinusitis (3/20),3 cases of nasal polyps (3/18),3 cases of inflammatory pseudotumor (3/17),4 cases of fungal sinusitis (4/20),1 case of rhinoscleroma (1/12),7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2).Conclusion Inflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon.Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings.Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum.IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.

  5. The clinical study on classification and diagnostic procedures of diffuse parenchymal lung disease/interstitial lung disease in 349 children%儿童弥漫性实质性肺疾病349例分类及诊断程序研究

    Institute of Scientific and Technical Information of China (English)

    张晶莹; 刘秀云; 彭芸; 周春菊

    2013-01-01

    Objective To better understand the spectrum of the disease and to explore the clinical significance of diagnostic procedures in clinical application. Method The medical records of 349 children with diffuse parenchymal lung disease /interstitial lung disease admitted to Beijing Children's hospital from July 2001 to September 2011 were studied retrospectively. The diagnostic approaches were applied, including the history, physical examination, non-invasive examination and invasive examination. Result The 321 cases were made specific diagnosis through the diagnostic procedures and 28 cases were unclassified. The classification of the 349 cases were as follows:146 cases were with primary lung diseases, 14 cases were with lung disease secondary to systemic disease, 161 cases had the lung disease of known causes, and the 28 cases were unclassified. The 160/349(45.85%) cases were diagnosed based on the noninvasive examination, including all 147 cases of bronchiolitis obliterans, 3 cases of hypersensitive pneumonitis, 2 cases secondary to aspiration, 2 cases of systemic lupus erythematosus, 2 cases of juvenile rheumatoid arthritis, 1 case infected by CMV, 1 case of Langerhans cell histocytosis, 1 case of undifferentiated connective tissue disease and 1 case of Wegener's granulo-matosis. The others were diagnosed by invasive examination, including 2 cases of Langerhans cell histocytosis and 1 case of dermatomyositis by skin biopsies, 1 case of Langerhans cell histocytosis by lymphaden biopsy, all 113 cases of idiopathic pulmonary hemosiderosis, 2 cases of eosinophilic pneumonia and 2 cases of hypersensitive pneumonitis by bronchoalveolar lavage fluid; 40 cases were diagnosied by the lung biopsy, including 23 cases of idiopathic interstitial pneumonia. Conclusion Diffuse parenchymal lung disease in children is a heterogeneous group of respiratory disorders. Bronchiolitis obliterans, idiopathic pulmonary hemosiderosis and idiopathic interstitial pneumonia are the most common

  6. Wirksamkeit und Verträglichkeit von Cyclophosphamid bei Multipler Sklerose: Eine retrospektive Analyse

    OpenAIRE

    2008-01-01

    Cyclophosphamid (Endoxan) ist ein zytostatisches Medikament, welches wegen seiner immunsuppressiven Wirkung eine breite Anwendung in der Therapie systemischer Autoimmunerkrankungen findet. Es wird als Medikation bei schwerer chronisch-progressiver Multipler Sklerose empfohlen, um die weitere Progredienz einzuschränken oder zu verhindern. Bisherige klinische Studien über den Wert dieses therapeutischen Einsatzes liefern aber kontroverse Ergebnisse. Aus diesem Grund erschien es sinnvoll...

  7. Coeliakie, gluten en de ontwikkeling van granen met verlaagde toxiciteit

    NARCIS (Netherlands)

    Gilissen, L.J.W.J.; Meer, van der I.M.; Smulders, M.J.M.

    2009-01-01

    Coeliakie is een voedingsgerelateerd probleem, veroorzaakt door een overgevoeligheidsreactie op gluten. De diagnose is moeilijk te stellen wegens de grote verscheidenheid aan symptomen. Een levenslang glutenvrij dieet is tot op heden de enige remedie. Gluten is echter een product dat in toenemende

  8. Fracturen van het distale deel van het femur

    NARCIS (Netherlands)

    Duyn, Cornelius Diederikus van

    1977-01-01

    ln dit proefschrift wordt een analyse gegeven van de behandeling en de daarmee bereikte resultaten van 123 patiënten die in de periode 1 januari 1958 tot en met 31 december 1972 wegens een fractuur van het distale deel van het femur werden behandeld in de Kliniek voor Heelkunde van het Academisch

  9. Ontwikkeling van licht reflecterende deklagen door KWS: KonweBright

    NARCIS (Netherlands)

    ter Huerne, Henderikus L.; Hetebrij, Dirk; Elfring, Jan

    2014-01-01

    In het kader van het milieu staan op dit moment asfalt verhardingsmaterialen met sterker lichtreflecterende eigenschappen in de belangstelling. Dergelijke materialen hebben als voordeel dat door de verbeterde reflectie-eigenschappen op de wegen minder openbare verlichting nodig is en toch een goede

  10. Receptivity of a Cryogenic Coaxial Gas-Liquid Jet to Acoustic Disturbances (Briefing Charts)

    Science.gov (United States)

    2014-03-01

    Distribution Unlimited 13. SUPPLEMENTARY NOTES Briefing Charts presented at 50th AIAA /ASME/SAE/ASEE Joint Propulsion Conference, Cleveland, OH, 28-30 July...of a Cryogenic Coaxial Gas-Liquid Jet to Acoustic Disturbances 50th AIAA /ASME/SAE/ASEE Joint Propulsion Conference Jeff Wegener, UCLA David Forliti

  11. Natuur- en milieu-educatie, tussen beleven en overleven : een cultuurpedagogisch vraagstuk

    NARCIS (Netherlands)

    Praamsma, J.M.

    1993-01-01

    Er zijn in de natuur- en milieu-educatie twee wegen te onderkennen. De eerste weg vertrekt vanuit de beleving van de natuur, de andere vanuit de wetenschappelijk-technische oplossing van milieuproblemen. In mijn bijdrage wil ik duidelijk maken dat binnen de natuur- en milieu-educatie deze beide bena

  12. ESA CryoVEx 2011

    DEFF Research Database (Denmark)

    Skourup, Henriette; Barletta, Valentina Roberta; Einarsson, Indriði

    data obtained with an airborne electromagnetic (AEM) induction sounder conducted by Alfred Wegener Institute (AWI) with fixed‐wing airplane (Polar‐5, Basler BT‐67). DTU Space airborne team visited five main validation sites: Devon ice cap (Canada), Austfonna ice cap (Svalbard), the EGIG line crossing...

  13. Parnassiana nova : XXXVI. Nachträgliche Betrachtungen zu der Revision der Subfamilie Parnassiinae (Fortsetzung 9)

    NARCIS (Netherlands)

    Eisner, C.

    1964-01-01

    Parnassius phoebus L. subsp. uralensis Ménétriés Bei Erwähnung dieser Unterart in Parnassiana 3: 56 — irrtümlich Stichel als Autor zugeschrieben — haben Bryk und ich erklärt, wegen des uneinheitlichen habitus der uns bekannten Exemplare von einer Diagnose Abstand nehmen zu sollen. Der Erhalt einer

  14. Coeliakie, gluten en de ontwikkeling van granen met verlaagde toxiciteit

    NARCIS (Netherlands)

    Gilissen, L.J.W.J.; Meer, van der I.M.; Smulders, M.J.M.

    2009-01-01

    Coeliakie is een voedingsgerelateerd probleem, veroorzaakt door een overgevoeligheidsreactie op gluten. De diagnose is moeilijk te stellen wegens de grote verscheidenheid aan symptomen. Een levenslang glutenvrij dieet is tot op heden de enige remedie. Gluten is echter een product dat in toenemende m

  15. New machinery is required for fuel cells. Mechanical engineering must keep in pace with the trend; Wer Brennstoffzellen will, braucht andere Maschinen. Maschinenbau darf Entwicklung nicht verpassen

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    2003-07-01

    Nobody knows whether fuel cells will have had their breakthrough by 2010. In view of the consequences for mechanical engineering, however, producers should keep abreast of developments. [German] Ob sich die Brennstoffzelle bis 2010 etabliert, kann noch niemand sicher sagen. Wegen der Folgen fuer den Maschinenbau sollten sich die Unternehmen jedoch mit dieser Moeglichkeit rechtzeitig befassen. (orig.)

  16. A global high-resolution data set of ice sheet topography, cavity geometry and ocean bathymetry

    DEFF Research Database (Denmark)

    Schaffer, Janin; Timmermann, Ralph; Arndt, Jan Erik;

    2016-01-01

    of Nioghalvfjerdsfjorden Glacierand Zachariæ Isstrøm have been obtained from the data centres of Technical University of Denmark (DTU),Operation Icebridge (NASA/NSF), and Alfred Wegener Institute (AWI). For the Antarctic ice sheet/ice shelves,RTopo-2 largely relies on the Bedmap-2 product but applies corrections...

  17. CryoSat-2 Validation using CryoVEX 2011-12 Airborne Campaigns

    DEFF Research Database (Denmark)

    Skourup, Henriette; Forsberg, René; Kildegaard Rose, Stine;

    north of Alert and sea ice around Svalbard in the Fram Strait. Selected tracks were planned to match CryoSat-2 passes and a few of them were flown in formation flight with the Alfred Wegener Institute (AWI) Polar-5 carrying an EM-bird. This presentation summarizes the 2011-12 airborne campaigns...

  18. CryoVEx 2011-12 Airborne Campaigns for CryoSat Validation

    DEFF Research Database (Denmark)

    Skourup, Henriette; Hvidegaard, Sine Munk; Forsberg, René;

    2013-01-01

    of Alert and sea ice around Svalbard in the Fram Strait. Selected tracks were planned to match CryoSat-2 passes and a few of them were flown in formation flight with the AlfredWegener Institute (AWI) Polar- 5 carrying an EM induction sounder. The paper presents an overview of the 2011-12 airborne campaigns...

  19. A new numerical model for simulating the propagation of and inundation by tsunami waves

    NARCIS (Netherlands)

    Cui, H.

    2013-01-01

    This thesis has involved the development of an unstructured grid ocean model, H2Ocean, with accurate flooding and drying algorithms for tsunami studies. The research is co-funded by the Alfred Wegener Institute (AWI) in Bremerhaven, Germany, as part of their contribution to the German-Indonesian Tsu

  20. Inzetbaarheid van RF en ICP-MS voor de vaststelling van metaalgehalten in verpakkingsmateriaal : Regeling Verpakking en Verpakkingsafval

    NARCIS (Netherlands)

    van Dijk J; van de Beek A; Ritsema R; LAC

    2003-01-01

    In het kader van de "Regeling Verpakking en Verpakkingsafval" worden eisen gesteld aan de gehalten van cadmium, lood, kwik en zeswaardige chroom. De som van de gehalten mag de grens van 100 ug/g niet overschrijden. Wegens analytische beperkingen wordt chroom als totaal chroom (Cr)

  1. Alles was wir sehen, ist ein Bild : Philipp Otto Runge in het licht van de vroeg-romantische poëzietheorie van Friedrich Schlegel en Novalis

    NARCIS (Netherlands)

    Kintz, P.A.M.

    2009-01-01

    Philipp Otto Runge geldt als een van de radicaalste vernieuwers van de Duitse schilderkunst aan het begin van de 19de eeuw. De reden: hij brak met de heersende classicistische traditie en zocht naar nieuwe wegen voor de kunst. Échte kunst, zei Runge, is als religie: zij is spreekbuis van het onzicht

  2. Halt: een alternatieve aanpak van vandalisme : Eindrapport van een evaluatie-onderzoek naar Halt-projecten

    NARCIS (Netherlands)

    Kruissink, M.; Verwers, C.

    1989-01-01

    Doel: HALT-projecten hebben tot doel het vandalisme te bestrijden. Deze projecten zijn ontstaan uit een samenwerking van gemeente, politie en justitie. Strafrechtelijk minderjarigen die wegens vandalisme zijn aangehouden kunnen door de politie of de Officier van Justitie naar HALT verwezen worden. V

  3. Inzetbaarheid van RF en ICP-MS voor de vaststelling van metaalgehalten in verpakkingsmateriaal : Regeling Verpakking en Verpakkingsafval

    NARCIS (Netherlands)

    van Dijk J; van de Beek A; Ritsema R; LAC

    2003-01-01

    In het kader van de "Regeling Verpakking en Verpakkingsafval" worden eisen gesteld aan de gehalten van cadmium, lood, kwik en zeswaardige chroom. De som van de gehalten mag de grens van 100 ug/g niet overschrijden. Wegens analytische beperkingen wordt chroom als totaal chroom (Cr) bepaal

  4. Predictie gronddeformaties: case Betuweroute km 16.7

    NARCIS (Netherlands)

    Feddema, A.; Rietdijk, J.

    2005-01-01

    Dit rapport maakt deel uit van het onderzoek naar omgevingsbeïnvloeding bij ophogingen en wegverbredingen in het kader van het DelftCluster programma Blijvend Vlakke Wegen. Binnen dit onderzoek wordt onderzoek gedaan naar de voorspellende waarde met betrekking tot de zettingen en horizontale vervorm

  5. The "Blue Banana" Revisited

    NARCIS (Netherlands)

    Faludi, A.K.F.

    2015-01-01

    This essay is about the “Blue Banana”. Banana is the name given subsequently by others to a Dorsale européenne (European backbone) identified empirically by Roger Brunet. In a background study to the Communication of the European Commission ‘Europe 2000’, Klaus Kunzmann and Michael Wegener put forwa

  6. ProMeV - Proactief Meten van Verkeersveiligheid : inzicht in onveiligheid vóórdat er slachtoffers vallen.

    NARCIS (Netherlands)

    Aarts, L.T. Dijkstra, A. & Bax, C.A.

    2014-01-01

    ProMeV helpt beleidsmakers keuzen te maken voor de inrichting van hun wegen en wegennetwerk zonder daarbij afhankelijk te zijn van gegevens over ongevallen. ProMeV is een proactief instrument dat is ontwikkeld in opdracht van het Interprovinciaal Overleg (IPO). Het bevat vooralsnog de volgende metho

  7. A new numerical model for simulating the propagation of and inundation by tsunami waves

    NARCIS (Netherlands)

    Cui, H.

    2013-01-01

    This thesis has involved the development of an unstructured grid ocean model, H2Ocean, with accurate flooding and drying algorithms for tsunami studies. The research is co-funded by the Alfred Wegener Institute (AWI) in Bremerhaven, Germany, as part of their contribution to the German-Indonesian

  8. Over het belang van Temminck's „Discours préliminaire" voor de zoologische Nomenclatuur

    NARCIS (Netherlands)

    Mees, G.F.

    1957-01-01

    Het gewoonlijk als „Discours préliminaire" aangeduide artikel van Temminck heeft nimmer die aandacht van dierkundigen getrokken, die het, wegens zijn systematisch belang, verdient. Ten dele vindt dit stellig zijn oorzaak in de betrekkelijke zeldzaamheid van het werk, ten dele ook in het feit dat het

  9. Over het belang van Temminck's „Discours préliminaire" voor de zoologische Nomenclatuur

    NARCIS (Netherlands)

    Mees, G.F.

    1957-01-01

    Het gewoonlijk als „Discours préliminaire" aangeduide artikel van Temminck heeft nimmer die aandacht van dierkundigen getrokken, die het, wegens zijn systematisch belang, verdient. Ten dele vindt dit stellig zijn oorzaak in de betrekkelijke zeldzaamheid van het werk, ten dele ook in het feit dat het

  10. Revised analysis of the (1+1) EA for the minimum spanning tree problem

    DEFF Research Database (Denmark)

    Witt, Carsten

    2014-01-01

    We revisit the classical analysis of the (1+1) EA for the minimum spanning tree problem in the case that nothing is known about the weights of the underlying graph. Here the original upper bound on the expected running time by Neumann and Wegener [Theor. Comput. Sci. 378(1), 32-40, 2007], which...

  11. [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.

  12. Genetically Modified T-cell Infusion Following Peripheral Blood Stem Cell Transplant in Treating Patients With Recurrent or High-Risk Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-27

    Adult Grade III Lymphomatoid Granulomatosis; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  13. Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas

    Science.gov (United States)

    2013-06-04

    Childhood Burkitt Lymphoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Liver Cancer; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway Glioma; Recurrent Colon Cancer; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Melanoma; Recurrent Nasopharyngeal Cancer; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  14. [Otolaryngologic manifestations of Churg-Strauss sindrome. Report of a case and review of the literature].

    Science.gov (United States)

    Pino Rivero, V; González Palomino, A; Pantoja Hernández, C G; Trinidad Ruíz, G; Pardo Romero, G; Montero García, C; Blasco Huelva, A

    2006-01-01

    Churg-Strauss Sindrome or allergic granulomatosis is a small vessel systemic vasculitis characterized by asthma, hypereosinophilia and necrotizing vasculitis with extravascular eosinophil granulomas. We describe a case assisted in our hospital who presented pulmonary infiltrates, fever, peripherical neuropathy, weight loss, myalgia, rhinosinusitis, with antecedents of nasal polyposis, and facial edema. Our aim is to review the main otolaryngologic manifestations of this rare illness that is treated with oral corticosteroids, immunosupresor drugs like ciclofosfamide and plasmapheresis.

  15. Asymptomatic endoalveolar hemorrhage in a young male

    Directory of Open Access Journals (Sweden)

    Alain Kafyeke

    2016-06-01

    Full Text Available We describe the case of a young male affected by granulomatosis with polyangiitis presenting with non-specific complaints and complicated by the occurrence of a diffuse endoalveolar hemorrhage characterized by atypical clinical and radiological features. The importance of a rapid and aggressive diagnostic and therapeutic approach has to be strongly underlined. Available data regarding prevalence, clinical and radiological characteristics and treatment of this uncommon manifestation have also been hereby reviewed.

  16. Genetically Distinct Subsets within ANCA-Associated Vasculitis

    Science.gov (United States)

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

    2013-01-01

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

  17. Lethal Midline Granuloma

    Directory of Open Access Journals (Sweden)

    Ghosh S.N

    1995-01-01

    Full Text Available Midline granuloma is a term with several synonyms: Stewart’s granuloma, granuloma gangraenescens nasi, lethal granuloma, polymorphous reticulosis 2-4 The conditiin is characterized by localized inflammation, destruction and often mutilation of tissues of the upper respiratory tract and face and, unless treated, it ends fatally within 12 to 18 months of onset. The pathological changes are those of nonspecific chronic inflammation to necrotizing vasculitis and granulomatosis. A case of lethal midline granuloma is reported.

  18. Documentation for the Computer Assisted Diagnostic Program for Dental Pain

    Science.gov (United States)

    1989-04-10

    palate) Amyloidosis *Sarcoidosis *Verruca vulgaris, mIultiple lesions Focal dermal hypcplasia syndrume Darier’s disease *Acanthosis nigricans * Crohn ’ s ...mouth) Mouth breathing *Diabetes (uncontrolled) *Weener s granulomatosis *Cyclic neutropenia *Cushing’s syrom *Yellow fever Scurvy Vitamin A deficiency...vulgaris, *Verucou carcincna *Xoplick spots (neasles) Verzucoas x0nthcmna Epidemid cyst Lyzrlioepithelial cyst Acanthosis nigricans *ai~er I s disease C-4

  19. [The development of Rein van Bemmelens (1904-1983) undation theory: forty years of Dutch geology].

    Science.gov (United States)

    Barzilay, Willemjan

    2009-01-01

    The Dutch geologist Rein van Bemmelen was the greatest opponent of plate tectonics in The Netherlands. He lived and worked during an important period in the history of earth sciences. He had studied geology when Wegeners theory was introduced and enthusiastically received in the Netherlands and he worked as a geologists during the period in which, after Wegeners theory was rejected in The Netherlands, several Dutch geologists came with their own theories to explain the origin of continents and oceans and in which plate tectonics was introduced in The Netherlands. He had proposed his own theory, the undation theory, at the beginning of the 1930s and kept on developing it during the following years. He continued to do so until his death in 1983. The history of the undation theory thus sheds light on the history of geology in The Netherlands. I will trace the history of geology in The Netherlands using Rein van Bemmelen and his undation theory as a lens.

  20. Standardisierung im E-Learning oder Vom schleichenden Untergang der Didaktik

    Directory of Open Access Journals (Sweden)

    Stefan Krause

    2002-10-01

    Full Text Available Die Autoren diskutieren die Vor- und Nachteile einer Standardisierung von E-Learning Formaten. Ähnlich wie der Standard Rich Text zum Datenaustausch von Textdateien standardisiert wurde, können Lerneinheiten (learning objects als standardisierte, wieder verwendbare Objekte gespeichert und vielseitig eingesetzt werden. Eine Möglichkeit besteht im Ablegen in zentralen Pools in Hochschulen, wo sie in unterschiedliche Plattformen eingebunden werden. Die Autoren stellen in Frage, ob hierbei wegen der Fokussierung auf Effizienz und Atomisierung von Information mediendidaktische Überlegungen vernachlässigt werden und der Begriff des Wissens reduziert wird auf abrufbare Informationen. In einem Exkurs verdeutlichen sie die Problematik am Beispiel von Studierenden der Medizin, die über ein gutes Faktenwissen verfügen, häufig aber Schwierigkeiten bei der Anwendung haben. In diesem Zusammenhang stellen sie die Kliniksimulation "Prometheus" vor, die wegen ihrer Handlungsorientierung die Studierenden zur Expertise anleiten soll.