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: Alexander Tang, David M.P. Di Fonzo, Saba Alsaddah, Chelsea Maedler-Kron, Arielle Mendel, Giada Sebastiani
Format: Article
Language:English
Published: American College of Physicians 2024-11-01
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.
ISSN:2767-7664