Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type

Abstract

Objective To evaluate whether the classification of ANCA-associated vasculitis (AAV) patients according to ANCA type (anti-proteinase 3 [PR3] or anti-myeloperoxidase [MPO] antibodies) predicts treatment response. Methods Treatment responses were assessed among patients enrolled in the Rituximab in ANCA-associated Vasculitis trial according to both AAV diagnosis (granulomatosis with polyangiitis [GPA]/microscopic polyangiitis [MPA]) and ANCA type (PR3-AAV/MPO-AAV). Complete remission (CR) was defined as disease activity score of 0 and successful completion of the prednisone taper. Results PR3-AAV patients treated with rituximab (RTX) achieved CR at 6 months more frequently than did those randomized to cyclophosphamide (CYC)/azathioprine (AZA) (65% versus 48%; P=0.04). The odds ratio (OR) for CR at 6 months among PR3-AAV patients treated with RTX as opposed to CYC/AZA was 2.11 (95%CI 1.04–4.30) in analyses adjusted for age, sex, and new-onset versus relapsing disease at baseline. PR3-AAV patients with relapsing disease achieved CR more often following RTX treatment at 6 months (OR3.57; 95%CI 1.43–8.93); 12 months (OR4.32; 95%CI 1.53–12.15); and 18 months (OR3.06; 95%CI 1.05–8.97). No association between treatment and CR was observed in the MPO-AAV patient subset or in groups divided according to AAV diagnosis. Conclusion PR3-AAV patients respond better to RTX than to CYC/AZA. An ANCA type-based classification may guide immunosuppression in AAV.

Disease Activity, Antineutrophil Cytoplasmic Antibody Type, and Lipid Levels in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

“…Their observations contrast with those reported in other inflammatory conditions . While these differences may reflect differences between clinical trial cohorts and clinic‐based cohorts, our findings are consistent with the preponderance of data on other inflammatory conditions and data from a prior cross‐sectional study evaluating CVD risk in a small AAV cohort .…”

“…conditions (5,25,26). While these differences may reflect differences between clinical trial cohorts and clinic-based cohorts, our findings are consistent with the preponderance of data on other inflammatory conditions (5,25,26) and data from a prior crosssectional study evaluating CVD risk in a small AAV cohort (21). Our findings add to a growing body of literature describing an inverse relationship between lipid levels and inflammation (5,25,26).…”

Difference in relapse-rate and clinical phenotype by autoantibody-subtype in Japanese patients with anti-neutrophil cytoplasmic antibody-associated vasculitis

“…The optimal maintenance therapy with rituximab, the effectiveness of rituximab to prevent relapse, and the safety of rituximab treatment in Japanese patients with AAV remain to be established. Classification by ANCA subtype may be useful to guide the use of immunosuppression in AAV because patients with PR-3 ANCA vasculitis responded rituximab better than cyclophosphamide [30].…”

ANCA-associated vasculitis

“…In the RAVE trial, a better response was seen in PR3 positive patients. 15 An alternative regimen of 1 gm given on two occasions 2 weeks apart has been widely used and shown to be as effective. 16 In the UK, rituximab may be used in AAV for primary induction in the following circumstances:…”