Wakamatsu Toru, Kanda Tatsuo, Tawada Akinobu, Miyamura Tatsuo, Takahashi Masanori, Chiba Tetsuhiro, Arai Makoto, Maruyama Hitoshi, Fujiwara Keiichi, Imazeki Fumio, Yokosuka Osamu
Department of Medicine and Clinical Oncology, Chiba University Graduate School of Medicine, Chiba, Japan.
Case Rep Gastroenterol. 2012 May;6(2):394-9. doi: 10.1159/000339915. Epub 2012 Jun 19.
Antimitochondrial antibody (AMA) is one of the representative features of primary biliary cirrhosis (PBC). PBC is a female-dominant disease usually presenting intrahepatic bile duct destruction, cholestasis and fibrosis with or without chronic nonsuppurative destructive cholangitis. We presented the case of a 63-year-old man with acute liver failure who had AMA, pronounced alanine aminotransferase elevation and high bilirubinemia. We administered corticosteroids and rescued this patient without liver transplantation. It is well known that some patients within the spectrum of autoimmune liver disease present with characteristics of both PBC and autoimmune hepatitis. Although corticosteroids may be associated with a significant worsening of adverse events in patients with PBC, if acute liver failure in AMA-positive cases is progressive, the administration of corticosteroids has to be considered, as well as the preparation of urgent liver transplantation.
抗线粒体抗体（AMA）是原发性胆汁性肝硬化（PBC）的典型特征之一。PBC是一种女性主导的疾病，通常表现为肝内胆管破坏、胆汁淤积和纤维化，伴有或不伴有慢性非化脓性破坏性胆管炎。我们报告了一例63岁男性急性肝衰竭患者，该患者存在AMA、显著的丙氨酸转氨酶升高和高胆红素血症。我们给予了皮质类固醇治疗，使该患者无需肝移植而获救。众所周知，一些自身免疫性肝病患者同时具有PBC和自身免疫性肝炎的特征。虽然皮质类固醇可能会使PBC患者的不良事件显著恶化，但如果AMA阳性病例的急性肝衰竭呈进行性发展，则必须考虑给予皮质类固醇治疗，同时做好紧急肝移植的准备。
