Acute-on-chronic liver failure in primary biliary cholangitis by exacerbation of autoimmune hepatitis features

We herein describe an autopsy case of acute-on-chronic liver failure (ACLF) in a patient with primary biliary cholangitis (PBC) that was triggered by an exacerbation of autoimmune hepatitis (AIH)-related features. A 62-year-old woman was diagnosed as having PBC 12 years prior and had been maintained on ursodeoxycholic acid. She presented with fatigue, and was found to have acute exacerbation of liver injury. Her liver function rapidly deteriorated. Upon transfer to our hospital, she exhibited marked hyperbilirubinemia, severe coagulopathy, and ascites, which fulfilled the ACLF diagnostic criteria of the Asian Pacific association for the study of the liver (APASL). Despite intensive treatment including plasma exchange, she died on day 15 of hospitalization. Autopsy revealed advanced hepatic fibrosis with lobular distortion, chronic non-suppurative destructive cholangitis, and loss of interlobular bile ducts, which were characteristic of PBC, along with centrilobular hepatic necrosis, bridging necrosis, bile ductular proliferation, and bile plugs that were suggestive of submassive hepatic necrosis. Interface hepatitis with plasma cell infiltration was also evident. These results indicated an overlap between chronic PBC and AIH exacerbation, leading to ACLF. Our findings highlight the importance of recognizing overlap features in autoimmune liver diseases for more timely interventions and better outcomes in these complex cases.