Kobayashi Yoshiaki, Iwadare Takanobu, Ida Masayuki, Miyatsu Chiaki, Yamashita Yuki, Yamazaki Tomoo, Uehara Kai, Uehara Takeshi, Nakanuma Yasuni, Kimura Takefumi
Department of Medicine, Division of Gastroenterology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.
Department of Medicine, Division of Nephrology, Shinshu University School of Medicine, Matsumoto, Japan.
Clin J Gastroenterol. 2025 Aug 5. doi: 10.1007/s12328-025-02198-9.
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.
我们在此描述一例原发性胆汁性胆管炎（PBC）患者发生慢加急性肝衰竭（ACLF）的尸检病例，该病例由自身免疫性肝炎（AIH）相关特征的加重所引发。一名62岁女性12年前被诊断为PBC，一直服用熊去氧胆酸治疗。她出现乏力症状，检查发现肝损伤急性加重，肝功能迅速恶化。转至我院时，她表现出明显的高胆红素血症、严重的凝血功能障碍和腹水，符合亚太肝脏研究协会（APASL）的ACLF诊断标准。尽管接受了包括血浆置换在内的强化治疗，但她在住院第15天死亡。尸检显示有进展性肝纤维化伴小叶结构紊乱、慢性非化脓性破坏性胆管炎以及小叶间胆管缺失，这些是PBC的特征，同时还有中心性肝坏死、桥接坏死、胆小管增生和胆栓，提示亚大块肝坏死。界面性肝炎伴浆细胞浸润也很明显。这些结果表明慢性PBC与AIH加重之间存在重叠，导致了ACLF。我们的发现强调了认识自身免疫性肝病重叠特征对于在这些复杂病例中更及时地进行干预并取得更好预后的重要性。
