NDT Advance Access originally published online on June 22, 2004
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Nephrol Dial Transplant (2004) 19: 1964-1968
Nephrol Dial Transplant Vol. 19 No. 8 © ERA-EDTA 2004; all rights reserved
Editorial Review
Renal vasculitis—an update in 2004
1 Department of Medicine (Box 157), Addenbrooke's Hospital, Cambridge, UK and 2 Division of Medicine, Imperial College London, Hammersmith Hospital, London, UK
Correspondence and offprint requests to: Dr A. D. Booth. Department of Medicine, Box 157, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK. Email: tonybooth1@yahoo.co.uk
Keywords: ANCA; renal; vasculitis
| The first 150 words of the full text of this article appear below. |
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.
Epidemiology
The annual incidence and point prevalence of renal vasculitis in Europe is 10–20/million/year
Aetiology
Diagnosis
Pathogenesis
Outcome
Mortality
Renal outcome
Relapse
Therapy
Current treatment
Remission induction
Remission maintenance
Severe renal disease
Adverse events
Immunotherapy in renal vasculitis
Summary
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