Avacopan Causing Vanishing Bile Duct Syndrome in an Adult Patient With Microscopic Polyangiitis
Microscopic polyangiitis (MPA) is a systemic necrotizing small-vessel vasculitis that may affect multiple organs. Avacopan, an oral complement (C5a) receptor inhibitor, was recently approved and recommended as a glucocorticoid-sparing induction therapy for severe granulomatosis with polyangiitis and...
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| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
American College of Physicians
2024-11-01
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| Series: | Annals of Internal Medicine: Clinical Cases |
| Online Access: | https://www.acpjournals.org/doi/10.7326/aimcc.2024.0602 |
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| Summary: | Microscopic polyangiitis (MPA) is a systemic necrotizing small-vessel vasculitis that may affect multiple organs. Avacopan, an oral complement (C5a) receptor inhibitor, was recently approved and recommended as a glucocorticoid-sparing induction therapy for severe granulomatosis with polyangiitis and MPA. We present a case of a 74-year-old patient with MPA who was treated with avacopan and who developed a severe cholestatic liver injury. A liver biopsy showed histologic evidence of vanishing bile duct syndrome. This case highlights the importance of considering vanishing bile duct syndrome in patients who take avacopan. In this clinical context, prompt recognition and discontinuation of avacopan is essential to avoid worsening liver injury. |
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| ISSN: | 2767-7664 |