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Anca-Associated Vasculitides: Immunological and Clinical Aspects

Anca-Associated Vasculitides: Immunological and Clinical Aspects

Paperback

Series: Advances in Experimental Medicine and Biology, Book 336

Medical Reference

ISBN10: 1475791844
ISBN13: 9781475791846
Publisher: Springer Nature
Published: Jun 29 2013
Pages: 552
Weight: 2.18
Height: 1.17 Width: 7.00 Depth: 10.00
Language: English
WEGENER'S GRANULOMATOSIS & ANCA-ASSOCIATED DISEASES: THE STORY CONTINUES The disease now designated as Wegener's granulomatosis (WG) was first described in 1931 by Heinz Klinger, who considered it to be a special form of polyarteritis nodosa. Klinger's friend, Friedrich Wegener, expanded on the first observations and interpreted the pathological and clinical fmdings to represent a distinct disease entity (Wegener, 1939). He described this entity as a peculiar rhinogenous granulomatosis with a unique participation of the arterial system and the kidneys. Later, Godman and Churg (1954) established the classical diagnostic criteria (the WG triad): granuloma, vasculitis, and glomerulonephritis. In 1958 Walton pointed out the poor prognosis of WG based on a small number of published cases (mean survival time: 5 months). In 1966 Carrington and Liebow reported limited forms of WG with a defmitely more favorable prognosis. Since then positive results have been reported with cyclophosphamide therapy. In addition, a retrospective study of combined low-dose cyclophosphamide and prednisolone in 85 WG patients over a period of 21 years found a similarly encouraging outcome. The-latter experience led to the current standard treatment protocol (FAUCI et al., 1973 and 1983). More recently, strong evidence has emerged that some of the morbidity and mortality ofWG - and other types of systemic vasculitis - may be a consequence of this treatment (Hoffman et al., 1992).

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