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

Abstract

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.

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

“…In MPA, immune deposits on the vessel wall are usually absent, and serum antineutrophil cytoplasmic antibodies (ANCAs) are frequently present. MPA shares these characteristic features with other ANCA-associated vasculitides (AAVs) such as granulomatosis with polyangiitis (Wegener's) (GPA), eosinophilic GPA (Churg-Strauss), and renal limited vasculitis (1,2).…”

“…In patients with MPA, a wide range of organ systems can be affected (1)(2)(3). The lung is one of the major organs involved, and recent studies demonstrated that pulmonary involvement is associated with unfavorable outcomes (4,5).…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Long-term maintenance therapy may be achieved with a variety of immunomodulatory drugs, including methotrexate and azathioprine [150]. The survival rate for patients with WG is estimated to be 74 to 91% at 5 years [136,151].…”

“…In a recent study in which all patients were treated with high-dose corticosteroids to induce remission, rituximab was shown to be as good as cyclophosphamide for treating AAVs and better than cyclophosphamide for patients with relapsingremitting disease [147,148]. The survival rate for MPA is reported to be 45 to 76% at 5 years [136,151].…”

“…Rituximab has also been reported to improve outcome in both patients with and without CNS involvement [163,164]. The survival rate for CSS is reported to be 60 to 97% at 5 years [136,151].…”