Analysis of Clinical Outcomes in ANCA-Associated Glomerulonephritis Treated With Rituximab: A Single Center Experience in Japan

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Background: Contrary to many Western countries, MPO-AAV is dominant in Japan. The therapeutic response to rituximab (RTX) might differ. Therefore, we conducted a retrospective analysis of the clinical database of the 80 ANCA associated glomerulonephritis (ANCA-GN)-patients in our hospital. Methods: All patients met the CHCC classification criteria for MPA and GPA at disease onset. Eighty patients [53(66%) females)] followed for at least six months since 2014 (up to Mar 2022) were analyzed. Remission was defined as BVAS 0. We divided the ANCA-GN patients into the 4 groups [Group 1 (RTX induction+, maintenance+): 18 cases), Group 2 (RTX induction+, maintenance-): 13), Group 3 (immunosuppressant+): 23) and Group 4 (glucocorticoid only): 26)], and their clinical features and renal prognosis were compared. Results: Of the 80 patients (53 MPO-MPA, 21 MPO-GPA, and 6 PR3-GPA), patients [25 newly diagnosed or 6 relapsing diseases] received a remission induction with RTX therapy. The frequencies of RPGN in ANCA-GN were 69%, 72% in group 1, 85% in group 2, 22% in group 3 and 73% in group 4, respectively. The average ages at onset were 64.5 ± 15.6, 79.5 ± 5.1, 74.5 ± 8.1, 81.7 ± 5.5 years. The mean ages at the initiation of RTX were 65.8±14.8 in group 1, 80±5.2 in group 2, of which 33%, 92% were over 75 years. Maintenance therapy was given more often based on changes in CD19+ counts and/or ANCA titer in 81%, as compared to scheduled administration every 6 months. The BVAS were 14.3 ± 6.2, 17.9 ± 2.8, 12.2± 4.2, 13.8 ± 5.8. Serum creatinine levels (mg/dl) were 1.9 ± 1.1, 2.7 ± 1.5, 1.7 ± 1.8, 3.1 ± 2.7. Group 1 had high survival and renal survival, which was better than group 3. Most of the deaths in group 2 and 4 were within 1 year, and renal death had already occurred at the intervention within 3 months during induction therapy. Infection was the most common cause of death in all groups. In group 1 and 2, there was a tendency for recovery of renal function 1 year after the induction of remission, indeed, the dialysis withdrawal rates were 88%. The remission rates at 6/12 months were 85/77, 73/55, 92/67, 58/58%. The achievement rates of daily PSL dose of 10mg at 6 months/5mg at 12 months were 62/54, 64/45, 25/8, 26/11%. Conclusions: These results showed that RTX is effective and has an acceptable safety profile in relatively elder AAV-GN patients in daily practice.

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