Real-World Experience With Avacopan in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis

IntroductionPost-marketing data on outcomes of avacopan use in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are lacking.MethodsWe performed a multi-center retrospective analysis of 92 patients with newly diagnosed or relapsing AAV who received therapy with avacopan. The co-primary outcome measures were clinical remission at 26 and 52 weeks.. We use descriptive statistics and univariate logistic regression to assess outcomes and predictors of remission, respectively.ResultsOf the 92 patients, 21 (23%) had a baseline eGFR < 15 mL/min/1.73 m2 with 10% on kidney replacement therapy (KRT) at baseline. Among those with kidney involvement, mean (standard deviation) enrollment eGFR was 33 (27) mL/min/1.73 m2 with a mean change of +12 (25) and +20 ( 23) mL/min/1.73 m2 at weeks 26 and 52, respectively. In addition to avacopan, 47% of patients received combination therapy of rituximab and low-dose cyclophosphamide and 14% of patients received plasma exchange (PLEX). After induction, the median (interquartile range) time to start avacopan was 3.6 (2.1 - 7.7) weeks, and the median time to discontinue prednisone after starting avacopan was 5.6 (3.3 – 9.5) weeks. Clinical remission was achieved in 90% at week 26 and 84% at week 52. 20% of patients stopped avacopan due to adverse events, with the most common being elevated serum aminotransferases (4.3%).ConclusionA high rate of remission and an acceptable safety profile were observed with the use of avacopan in the treatment of AAV in this post-marketing analysis, including the populations excluded from the ADVOCATE trial.