@article {Unizony1166, author = {Sebastian Unizony and Miguel Villarreal and Eli M Miloslavsky and Na Lu and Peter A Merkel and Robert Spiera and Philip Seo and Carol A Langford and Gary S Hoffman and CG M Kallenberg and E William St. Clair and David Ikle and Nadia K Tchao and Linna Ding and Paul Brunetta and Hyon K Choi and Paul A Monach and Fernando Fervenza and John H Stone and Ulrich Specks}, title = {Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type}, volume = {75}, number = {6}, pages = {1166--1169}, year = {2016}, doi = {10.1136/annrheumdis-2015-208073}, publisher = {BMJ Publishing Group Ltd}, abstract = {Objective To evaluate whether the classification of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) 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 randomised to cyclophosphamide (CYC)/azathioprine (AZA) (65\% vs 48\%; p=0.04). The 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 to 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 (OR 3.57; 95\% CI 1.43 to 8.93), 12 months (OR 4.32; 95\% CI 1.53 to 12.15) and 18 months (OR 3.06; 95\% CI 1.05 to 8.97). No association between treatment and CR was observed in the MPO-AAV patient subset or in groups divided according to AAV diagnosis.Conclusions Patients with PR3-AAV respond better to RTX than to CYC/AZA. An ANCA type-based classification may guide immunosuppression in AAV.Trial registration number NCT00104299; post-results.}, issn = {0003-4967}, URL = {https://ard.bmj.com/content/75/6/1166}, eprint = {https://ard.bmj.com/content/75/6/1166.full.pdf}, journal = {Annals of the Rheumatic Diseases} }