Analysis of Clinical Features in ANCA-Associated Rapidly Progressive Glomerulonephritis Treated with Avacopan: A Single-Center Experience in Japan

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Background: Although avacopan (AVA) has recently become available as an induction therapy of ANCA-associated vasculitis, but the use of corticosteroid has not been established. Therefore, we conducted a retrospective analysis of the clinical database of ANCA-associated rapidly progressive glomerulonephritis (ANCA-RPGN)-patients in our hospital with a reduced regimen of corticosteroids. Methods: All patients met the CHCC classification criteria for MPA and GPA at disease onset. Seven patients [3(43%) females)] followed for at least 12 weeks since Aug 2022 (up to Apr 2023) were analyzed for clinical course, including remission rates. Remission was defined as Birmingham Vasculitis Activity Score (BVAS) 0. GC Pulse, RTX, and AVA were used for induction therapy. PSL was begun at a dose of 0.4-1.0 mg/kg/day and then tapered off at 12 weeks. Results: AVA was used in 12 patients with AAV, of whom 7 presented with RPGN. Of the 7 patients with renal involvement (4 MPO-MPA, 2 MPO-GPA and 1 PR3-GPA), 6 newly diagnosed and 1 relapsing disease received a remission induction. The mean age was 70.0±12.1 years, mean BVAS was 16.4±3.1, prednisolone (PSL) was 52.1±17.8 mg/day. At the start of AVA, s-Cr was 2.3±1.1 mg/dL, eGFR was 25.0±11.8 ml/min/1.73m2, CRP was 1.9±3.1 mg/dL and the dose of PSL was 37.1±17.0 mg/day. At week 12 and 24, percentages of participants who could discontinue PSL were 43%, 60% and remission rates were 100%, 80%, respectively. There were no serious adverse events and no patients requiring maintenance dialysis. At week 12 and 24, doses of PSL were 3.6±4.5/1.5±2.2 mg/day, s-Cr were 1.6±0.6 /1.7±0.7 mg/dL, eGFR were 34.2±14.2 /35.6±17.7 ml/min/1.73m2 and CRP were 2.7±6.7/0.11±0.09 mg/dl, respectively. Conclusions: These results showed that AVA may be effective and have acceptable safety profiles in relatively elder AAV-RPGN patients in daily practice in Japan.

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