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Anthony D. Booth, Charles D. Pusey, David R. Jayne, Renal vasculitis—an update in 2004, Nephrology Dialysis Transplantation, Volume 19, Issue 8, August 2004, Pages 1964–1968, https://doi.org/10.1093/ndt/gfh318
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Extract
1Department of Medicine (Box 157), Addenbrooke's Hospital, Cambridge, UK and 2Division of Medicine, Imperial College London, Hammersmith Hospital, London, UK
Introduction
The primary systemic vasculitides are a group of inflammatory diseases of unknown cause, which are usually fatal if untreated. Their classification has been harmonized over the last decade and an evidence base for treatment decisions is emerging. Current therapy saves lives, salvages organ function and reduces morbidity. However, mortality and end-stage renal failure (ESRF) rates remain high, up to 50% relapse and the consequences of treatment-related toxicity rival those of the underlying diseases. There is a need for safer, more effective therapy.
Classification
Primary systemic vasculitides are classified according to their clinical, serological and pathological features. The Chapel Hill Consensus conference defined diagnostic terminology in 1993 [1]. The largest subgroup comprises syndromes associated with circulating anti-neutrophil cytoplasm antibodies (ANCA)—ANCA-associated systemic vasculitides (AASV). These include Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss angiitis and renal limited vasculitis.
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