EXPERIENCE WITH AVACOPAN FOR THE TREATMENT OF ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS IN THE NEPHROLOGY DEPARTMENT OF NISHI-KOBE MEDICAL CENTER.

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https://storage.unitedwebnetwork.com/files/1099/de327e29c43145e3c54e6219e51559a6.pdf

Abstract Title *

EXPERIENCE WITH AVACOPAN FOR THE TREATMENT OF ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)-ASSOCIATED VASCULITIS IN THE NEPHROLOGY DEPARTMENT OF NISHI-KOBE MEDICAL CENTER.

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Co-author 1

Hiroko Kakita ponsuke318@yahoo.co.jp Nishi-Kobe Medical Center Nephrology Kobe Japan *

Co-author 2

Tomoki Suetake tomoki.sue.518@gmail.com Nishi-Kobe Medical Center Nephrology Kobe Japan -

Co-author 3

Kansei Otsuka Kyokui150018@gmail.com Nishi-Kobe Medical Center Nephrology Kobe Japan -

Co-author 4

Yosuke Nakata yosuke_nakata0617@yahoo.co.jp Nishi-Kobe Medical Center Nephrology Kobe Japan -

Co-author 5

Koichiro Hagihara hagi_hara@kuhp.kyoto-u.ac.jp Nishi-Kobe Medical Center Nephrology Kobe Japan -

Co-author 6

Co-author 7

Co-author 8

Co-author 9

Co-author 10

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Co-author 12

Co-author 13

Co-author 14

Co-author 15

Introduction

ANCA-associated vasculitis (AAV) is a rare, life-threatening disease that tends to develop in older individuals. Glucocorticoids (GCs) are effective in reducing disease activity; however, GC-related toxicity—such as infections, muscle weakness, and progression of arteriosclerosis—can significantly impair quality of life (QOL). In 2021, avacopan (AVA), a selective C5a receptor antagonist, was approved in Japan for the treatment of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) as a glucocorticoid-sparing therapeutic option.

Methods

We conducted a retrospective analysis of 20 patients with ANCA-associated vasculitis (AAV) who were treated with AVA for at least 26 weeks at the Department of Nephrology, Nishi-Kobe Medical Center. The primary outcomes were clinical remission and glucocorticoid dose reduction at 26 weeks. Descriptive statistics were used to evaluate the outcomes.

Results

A total of 26 patients received AVA treatment; 5 discontinued therapy (2 due to patient request, 1 due to elevated liver enzymes, 1 due to edema, and 1 due to insufficient therapeutic effect). Thus, 20 patients were evaluated.
Among them, 85% had microscopic polyangiitis. The mean baseline age was 75 years (SD 12), 60% were female, the mean baseline eGFR was 30 (SD 19) mL/min/1.73 m², and mean baseline urinary protein was 1.6 (SD 2.0) g/gCr.
Thirty-five percent received induction therapy, 35% relapse therapy, and 30% maintenance therapy. Concomitant immunosuppressive agents included cyclophosphamide in 6 patients, rituximab in 2, azathioprine in 5, and mizoribine in 6; 2 patients underwent plasma exchange.
Clinical remission was achieved in 85% of patients at 26 weeks. The mean prednisolone dose decreased from 13 (SD 7) mg to 7 (SD 3) mg. Three patients experienced relapse, one of whom had poor adherence to avacopan.
Elevated liver enzymes occurred in 3 patients, which resolved after temporary discontinuation. Avacopan was restarted at a lower dose in all cases—one with concomitant ursodeoxycholic acid and another after alcohol abstinence. No infections requiring hospitalization were observed during the observation period

Conclusion

Avacopan appeared to be a safe and effective glucocorticoid-sparing therapeutic option for AAV. However, regular monitoring of liver function is warranted.

Kewords