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Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force

¹ Botnar Research Centre, University of Oxford, Oxford, UK
² Department of Nephrology, Addenbrooke’s Hospital, Cambridge, UK
³ Department of Rheumatology, University Hospital of Schleswig-Holstein, Lübeck, Germany
⁴ Department of Rheumatology, University of Birmingham, Birmingham, UK
⁵ Department of Internal Medicine, Hospital Clinic, Barcelona, Spain
⁶ Division of Clinical and Experimental Immunology, Maastricht University, Maastricht, The Netherlands
⁷ Department of Internal Medicine, University of Paris Descartes, Paris, France
⁸ Vasculitis Center, Boston University School of Medicine, Boston, Massachusetts, USA
⁹ Institute of Social Medicine, University Hospital of Schleswig-Holstein, Lübeck, Germany
¹⁰ Department of Rheumatology, Norfolk Hospital, Norfolk, UK
¹¹ United States Food and Drug Administration, Rockville, Maryland, USA
¹² Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

Correspondence to:
Dr R Luqmani, Botnar Research Centre, University of Oxford, Windmill Road, Oxford OX3 7LD, UK; raashid.luqmani{at}noc.anglox.nhs.uk

Objectives: We undertook a systematic literature review as a background to the European League Against Rheumatism (EULAR) recommendations for conducting clinical trials in anti-neutrophil cytoplasm antibody associated vasculitis (AAV), and to assess the quality of evidence for outcome measures in AAV.

Methods: Using a systematic Medline search, we categorised the identified studies according to diagnoses. Factors affecting remission, relapse, renal function and overall survival were identified.

Results: A total of 44 papers were reviewed from 502 identified by our search criteria. There was considerable inconsistency in definitions of end points. Remission rates varied from 30% to 93% in Wegener granulomatosis (WG), 75% to 89% in microscopic polyangiitis (MPA) and 81% to 91% in Churg–Strauss syndrome (CSS). The 5-year survival for WG, MPA and CSS was 74–91%, 45–76% and 60–97%. Relapse (variably defined) was common in the first 2 years but the frequency varied: 18% to 60% in WG, 8% in MPA, and 35% in CSS. The rate of renal survival in WG varied from 23% at 15 months to 23% at 120 months. Methods used to assess morbidity varied between studies. Ignoring the variations in definitions of the stage of disease, factors influencing remission, relapse, renal and overall survival included immunosuppressive therapy used, type of organ involvement, presence of ANCA, older age and male gender.

Conclusions: Factors influencing remission, relapse, renal and overall survival include the type of immunosuppressive therapy used, pattern of organ involvement, presence of ANCA, older age and male gender. Methodological variations between studies highlight the need for a consensus on terminology and definitions for future conduct of clinical studies in AAV.