Vanishing Bile Duct Syndrome related to Avacopan Therapy in a Patient with ANCA-Associated Vasculitis.

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Vanishing Bile Duct Syndrome related to Avacopan Therapy in a Patient with ANCA-Associated Vasculitis.

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Co-author 1

Masatora Yamasaki sn5260@cc.saga-u.ac.jp Saga University Faculty of Medicine Saga Japan *

Co-author 2

Makoto Fukuda f8799@cc.saga-u.ac.jp Saga University Faculty of Medicine Saga Japan -

Co-author 3

Kohei Hashimoto sr1577@cc.saga-u.ac.jp Saga University Faculty of Medicine Saga Japan -

Co-author 4

Eriko Nonaka sp6686@cc.saga-u.ac.jp Saga University Faculty of Medicine Saga Japan -

Co-author 5

Maki Yoshihara st1320@cc.saga-u.ac.jp Saga University Faculty of Medicine Saga Japan -

Co-author 6

Motoaki Miyazono miyazono@cc.saga-u.ac.jp Saga University Faculty of Medicine Saga Japan -

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Introduction

ANCA-associated vasculitis is a disease that causes rapidly progressive glomerulonephritis. Avacopan, a C5a receptor inhibitor, is used as a new treatment for ANCA-associated vasculitis. However, in recent years, reports of drug-induced liver injury (DILI), including vanishing bile duct syndrome(VBDS), have been sporadically observed.

Methods

We report a 73-year-old woman. She presented with fatigue and was examined by a local physician, who noted elevated CRP, renal impairment, and pulmonary lesions. She was transferred to our hospital after testing positive for MPO-ANCA. A renal biopsy revealed crescentic glomerulonephritis, leading to a diagnosis of ANCA-associated vasculitis. Following steroid pulse therapy, she started oral prednisolone 40 mg daily, combined with avacopan. At a post-discharge outpatient visit (day 32 of avacopan administration), marked elevation of liver enzymes was noted, requiring readmission.

Results

The patient was diagnosed with liver injury due to abacavir and abacavir was discontinued. Persistent elevated bilirubin levels led to a liver biopsy, which confirmed VBDS. Treatment with plasma exchange resulted in improvement of the liver injury, and the patient was discharged.

Conclusion

To avoid progression to severe liver dysfunction, strict monitoring of liver function is required during treatment. Plasma exchange may be useful for improving liver dysfunction caused by avacopan.We presented atThe 55th Western Regianal Meeting of the Japanese Society of Nephrology.

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