Wegener's granulomatosis: A rare, chronic and multisystem disease
Wegener's granulomatosis (WG) is an autoimmune disease which has a clinical predilection for the upper airways, lungs and kidneys. It is a necrotising granulomatous vasculitis which is associated with a distinct autoantibody - the antineutrophil cytoplasmic antibody (ANCA). A heightened index o...
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Veröffentlicht in: | The surgeon (Edinburgh) 2006-02, Vol.4 (1), p.45-52 |
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description | Wegener's granulomatosis (WG) is an autoimmune disease which has a clinical predilection for the upper airways, lungs and kidneys. It is a necrotising granulomatous vasculitis which is associated with a distinct autoantibody - the antineutrophil cytoplasmic antibody (ANCA). A heightened index of suspicion by clinicians is needed in the diagnosis of this complex and rare condition. A multi-disciplinary approach should then be used to treat this chronic multisystem disease. Treatment involves the use of various regimens of corticosteroids and immunosuppressive medication. Mortality due to WG has been significantly decreased by this therapy. In this article, we focus on clinical manifestations and review the salient histologic, laboratory and serologic features and treatment |
doi_str_mv | 10.1016/S1479-666X(06)80021-1 |
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It is a necrotising granulomatous vasculitis which is associated with a distinct autoantibody - the antineutrophil cytoplasmic antibody (ANCA). A heightened index of suspicion by clinicians is needed in the diagnosis of this complex and rare condition. A multi-disciplinary approach should then be used to treat this chronic multisystem disease. Treatment involves the use of various regimens of corticosteroids and immunosuppressive medication. Mortality due to WG has been significantly decreased by this therapy. 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It is a necrotising granulomatous vasculitis which is associated with a distinct autoantibody - the antineutrophil cytoplasmic antibody (ANCA). A heightened index of suspicion by clinicians is needed in the diagnosis of this complex and rare condition. A multi-disciplinary approach should then be used to treat this chronic multisystem disease. Treatment involves the use of various regimens of corticosteroids and immunosuppressive medication. Mortality due to WG has been significantly decreased by this therapy. In this article, we focus on clinical manifestations and review the salient histologic, laboratory and serologic features and treatment</description><subject>Antibodies, Antineutrophil Cytoplasmic - immunology</subject><subject>antineutrophil cytoplasmic antibody</subject><subject>autoimmune</subject><subject>Biological and medical sciences</subject><subject>Biopsy, Needle</subject><subject>Chronic Disease</subject><subject>Disease Progression</subject><subject>Female</subject><subject>General aspects</subject><subject>Granulomatosis with Polyangiitis - epidemiology</subject><subject>Granulomatosis with Polyangiitis - immunology</subject><subject>Granulomatosis with Polyangiitis - pathology</subject><subject>Humans</subject><subject>Immunohistochemistry</subject><subject>Male</subject><subject>Medical sciences</subject><subject>multisystem</subject><subject>Prognosis</subject><subject>Risk Assessment</subject><subject>Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. 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Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis</topic><topic>Survival Analysis</topic><topic>Wegener's granulomatosis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Khan, A.M.</creatorcontrib><creatorcontrib>Elahi, F.</creatorcontrib><creatorcontrib>Hashmi, S.R.</creatorcontrib><creatorcontrib>Mahida, K.H.</creatorcontrib><creatorcontrib>Ingrams, D.R.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>The surgeon (Edinburgh)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Khan, A.M.</au><au>Elahi, F.</au><au>Hashmi, S.R.</au><au>Mahida, K.H.</au><au>Ingrams, D.R.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Wegener's granulomatosis: A rare, chronic and multisystem disease</atitle><jtitle>The surgeon (Edinburgh)</jtitle><addtitle>Surgeon</addtitle><date>2006-02-01</date><risdate>2006</risdate><volume>4</volume><issue>1</issue><spage>45</spage><epage>52</epage><pages>45-52</pages><issn>1479-666X</issn><eissn>2405-5840</eissn><abstract>Wegener's granulomatosis (WG) is an autoimmune disease which has a clinical predilection for the upper airways, lungs and kidneys. 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subjects | Antibodies, Antineutrophil Cytoplasmic - immunology antineutrophil cytoplasmic antibody autoimmune Biological and medical sciences Biopsy, Needle Chronic Disease Disease Progression Female General aspects Granulomatosis with Polyangiitis - epidemiology Granulomatosis with Polyangiitis - immunology Granulomatosis with Polyangiitis - pathology Humans Immunohistochemistry Male Medical sciences multisystem Prognosis Risk Assessment Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Survival Analysis Wegener's granulomatosis |
title | Wegener's granulomatosis: A rare, chronic and multisystem disease |
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