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Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss Syndrome)

  • Christian PagnouxEmail author
  • Loïc Guillevin
Chapter

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA; Churg–Strauss syndrome) is a rare, systemic, necrotizing, small-sized vessel vasculitis. It most typically affects middle-aged individuals who have developed late-onset asthma then more characteristic vasculitis manifestations, like fever, skin lesions (40–75 % of the patients, mainly purpura, often necrotic, and/or cutaneous nodules or papules and sometimes migratory urticarial rashes) and/or mononeuritis multiplex. Blood and tissue eosinophilia, especially the lungs, gastrointestinal tract, and heart, is a hallmark of the disease. Biopsy of an affected organ or lesions, especially skin lesions, can help in making the diagnosis, when showing eosinophilic and/or granulomatous vasculitis, with or without necrosis. Antineutrophil cytoplasm autoantibodies (ANCA) can be detected in nearly 40 % of the patients. Differential diagnoses include drug-induced and hypersensitivity vasculitis, parasitic infections, and hypereosinophilic syndromes. Treatment relies on systemic corticosteroids, combined with an immunosuppressant for severe and/or life-threatening forms.

Keywords

Erythema Multiforme Hypereosinophilic Syndrome Necrotizing Vasculitis Strauss Syndrome Eosinophilic Fasciitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  1. 1.Division of RheumatologyMount Sinai Hospital/University Health Network, The Rebecca MacDonald Centre for Arthritis and AutoimmunityTorontoCanada
  2. 2.Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Necrotizing Vasculitides and Systemic Sclerosis, Hôpital Cochin, Assistance Publique–Hôpitaux de ParisUniversité Paris DescartesParis Cedex 14France

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