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Books > Medicine > Clinical & internal medicine > Haematology
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ANCA-Associated Vasculitides - Immunological and Clinical Aspects (Paperback, Softcover reprint of the original 1st ed. 1993)
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ANCA-Associated Vasculitides - Immunological and Clinical Aspects (Paperback, Softcover reprint of the original 1st ed. 1993)
Series: Advances in Experimental Medicine and Biology, 336
Expected to ship within 10 - 17 working days
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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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