| 초록 |
Objectives: Antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (GN) responds rapidly to conventional immunosuppressive therapy but is frequently accompanied by treatment-related morbidities. Avacopan, a selective C5a receptor inhibitor, has emerged as a treatment option for immune-mediated GN involving the alternative complement pathway. This study aims to evaluate the efficacy and safety of avacopan in the Korean cohort with ACNA-GN. Methods: This multicenter cohort study included 12 patients with biopsy-proven ACNA-GN who received avacopan between 2024 and 2025. Primary outcomes were temporal changes in estimated glomerular filtration rate (eGFR) and proteinuria during the first year, and dialysis dependency. Results: The median duration of avacopan use was 334 days (interquartile range (IQR) 224–475). The mean eGFR level was 18.6 ± 10.8 ml/min/1.73m2 and the mean urine protein-to-creatinine ratio (UPCR) was 2.9 ± 3.1 g/g. The mean percentage of global glomerulosclerosis was 20.9%. Three and two patients belonged to the sclerotic and focal classes of the Berden classification, respectively. The changes in eGFR from baseline to 26 and 52 weeks were 9.7 ± 10.2 and 12.4 ± 9.4 ml/min/1.73m2, respectively. The changes in UPCR from baseline at 26 and 52 weeks were -1.9 ± 3.0 and -2.5 ± 3.2 g/g, respectively. A total of 5 patients initiated kidney replacement therapy (KRT), but 2 discontinued it within 3 months of initiation. The median duration of glucocorticoid use was 103 days (IQR 68–394), and 7 patients discontinued glucocorticoid within 3 months of initiation. Two patients experienced adverse effects of elevated liver enzyme levels related to avacopan, but these did not lead to the discontinuation of avacopan. Conclusion: This study highlights that favorable kidney outcomes were achieved with avacopan in the treatment of ANCA-GN, without the development of fatal adverse effects. Kidney function improved consistently throughout the treatment despite the early glucocorticoid withdrawal. |