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During the congress, E-Posters will be accessible to all participants on the congress website 24/7, as well as in the E-poster stations in the congress center.
Preparing your E-Poster
Please review the E-Poster format requirements carefully when preparing your E-Poster. Should your E-Poster not meet the mentioned requirements, it may not be displayed as described above.
E-Poster Submission Deadline
Please prepare and upload your E-Poster no later than March 14, 2026 11.59PM CET. After this date, you will no longer be able to prepare and upload your E-poster and it will not be displayed and accessible on the congress website.
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Abstract titles should be brief and reflect the content of the abstract.
【Background】Avacopan, a complement C5a receptor inhibitor, has emerged as a novel therapeutic option for ANCA-associated vasculitis (AAV), with well-established efficacy. Drug-induced liver injury (DILI) is the most common adverse event associated with avacopan. Recently, avacopan-induced vanishing bile duct syndrome (VBDS) has been reported, and some cases may progress to severe liver injury. However, its pathogenesis and optimal management remain unclear. Here, we report two cases of VBDS that developed after initiation of avacopan therapy for AAV.
【Case】The first case involved a 75-year-old man with rapidly progressive glomerulonephritis (RPGN) associated with AAV. After remission induction therapy with glucocorticoids, rituximab, and plasma exchange, avacopan (60 mg daily) was initiatedto facilitate rapid tapering of steroids. Jaundice developed 7 weeks after starting avacopan, accompanied by marked elevations in bilirubin and hepatobiliary enzymes. Imaging studies excluded extrahepatic biliary obstruction, and a subsequent liver biopsy revealed bile duct loss and lymphocytic infiltration in the portal tract, consistent with VBDS. On the basis of the clinical course, drug-induced VBDS due to avacopan was diagnosed. Despite discontinuation of avacopan and initiation of ursodeoxycholic acid (UDCA), liver dysfunction persisted, his general condition consequently worsened, and he ultimately died. The second case involved an 80-year-old woman who had acheived remission of AAV-related RPGN seven years earlier with glucocorticoid monotherapy, which was gradually tapered thereafter. Four months ago, relapse of AAV was suspected based on a rise in MPO-ANCA titers, and a low dose of avacopan (20 mg daily) was initiated to minimize the risk of hepatotoxicity. Four weeks after its initiation, she developed fatigue and laboratory findings showed marked elevations in bilirubin and hepatobiliary enzymes. Imaging studies again ruled out extrahepatic biliary obstruction and liver biopsy demonstrated severe bile duct loss, consistent with VBDS. Following discontinuation of avacopan and administration of UDCA, hepatobiliary enzymes levels gradually returned to the normal range.
【Discussion】It has been reported that the incidence of avacopan-related DILI is markedly higher in Japanese patients than in Western populations. VBDS associated with avacopan is a very rare but potentially severe form of DILI. Among our two cases of avacopan-related VBDS, one patient recovered after discontinuation of avacopan, whereas the other progressed to severe liver injury leading to death.
【Conclusion】Because avacopan-related VBDS can progrese to severe DILI, careful monitoring of liver function is essential after initiation of avacopan.
