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Outcomes from studies of Antineutrophil Cytoplasm Antibody Associated Vasculitis: a systematic review by the EULAR Systemic Vasculitis Task Force
  1. Chetan Mukhtyar (chetan.mukhtyar{at}
  1. University of Oxford, United Kingdom
    1. Oliver Flossmann (oflossmann{at}
    1. Addenbrooke's Hospital, United Kingdom
      1. Bernhard Hellmich (bernhard.hellmich{at}
      1. University of Schleswig Holstein, Campus Lübeck, Germany
        1. Paul A Bacon (p.a.bacon{at}
        1. University of Birmingham, United Kingdom
          1. Maria C Cid (mccid{at}
          1. Hospital Clínic, Spain
            1. Jan Willem Cohen Tervaert (secretariaat-immuno{at}
            1. Academic Hospital Maastricht, Netherlands
              1. Wolfgang L Gross (gross{at}
              1. Medizinische Universität Lubeck and, Germany
                1. Loic Guillevin (loic.guillevin{at}
                1. University of Paris Descartes, France
                  1. David RW Jayne (dj106{at}
                  1. Addenbrooke's Hospital, United Kingdom
                    1. Alfred Mahr (amahr{at}
                    1. Boston University, United States
                      1. Peter A Merkel (pmerkel{at}
                      1. Boston University, United States
                        1. Heiner Raspe
                        1. University of Schleswig-Holstein, Germany
                          1. David G I Scott (david.scott{at}
                          1. Norfolk & Norwich University Hospital, United Kingdom
                            1. James Witter (witterj{at}
                            1. FDA, United States
                              1. Hasan Yazici (hyazici{at}
                              1. TR-Kadikoy, Turkey
                                1. Raashid A Luqmani (raashid.luqmani{at}
                                1. Nuffield Orthopaedic Centre, United Kingdom


                                  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.

                                  • Antineutrophil Cytoplasm Antibody
                                  • Churg-Strauss syndrome
                                  • Wegener's granulomatosis
                                  • mMicroscopic polyangiitis
                                  • vasculitis

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