Outcomes from studies of Antineutrophil Cytoplasm Antibody Associated Vasculitis: a systematic review by the EULAR Systemic Vasculitis Task Force

Chetan Mukhtyar ¹, Oliver Flossmann ², Bernhard Hellmich ³, Paul A Bacon ⁴, Maria C Cid ⁵, Jan Willem Cohen Tervaert ⁶, Wolfgang L Gross ⁷, Loic Guillevin ⁸, David RW Jayne ², Alfred Mahr ⁹, Peter A Merkel ⁹, Heiner Raspe ¹⁰, David G I Scott ¹¹, James Witter ¹², Hasan Yazici ¹³ and Raashid A Luqmani ^14*

¹ University of Oxford, United Kingdom
² Addenbrooke's Hospital, United Kingdom
³ University of Schleswig Holstein, Campus Lübeck, Germany
⁴ University of Birmingham, United Kingdom
⁵ Hospital Clínic, Spain
⁶ Academic Hospital Maastricht, Netherlands
⁷ Medizinische Universität Lubeck and, Germany
⁸ University of Paris Descartes, France
⁹ Boston University, United States
¹⁰ University of Schleswig-Holstein, Germany
¹¹ Norfolk & Norwich University Hospital, United Kingdom
¹² FDA, United States
¹³ TR-Kadikoy, Turkey
¹⁴ Nuffield Orthopaedic Centre, United Kingdom

^* To whom correspondence should be addressed. E-mail: raashid.luqmani{at}noc.anglox.nhs.uk.

Accepted 23 September 2007

Abstract

Objectives: We undertook a systematic literature review as a background to the 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: 44 papers were reviewed, out of 502 identified by our search criteria. There was considerable inconsistency in definitions of end points. Remission rates varied from 30% to 93% in Wegener’s 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, 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 include: 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.

Keywords: Antineutrophil Cytoplasm Antibody, Churg-Strauss syndrome, Wegener's granulomatosis, mMicroscopic polyangiitis, vasculitis

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