Mendizabal Manuel, Marciano Sebastián, Videla María G, Anders Margarita, Zerega Alina, Balderramo Domingo C, Tisi Baña Matías R, Barrabino Martín, Gil Octavio, Mastai Ricardo, Yantorno Silvina, Gadano Adrián, Silva Marcelo O
aHepatology and Liver Transplant Unit bDepartment of Medicine, Hospital Universitario Austral cLiver Unit, Hospital Italiano de Buenos Aires dHepatology and Liver Transplant Unit, Fundación Favaloro eHepatology and Liver Transplant Unit, Hospital Alemán, Buenos Aires fLiver Transplant Unit, Sanatorio Allende gHepatology and Liver Transplant Unit, Hospital Privado de Córdoba, Córdoba, Argentina.
Eur J Gastroenterol Hepatol. 2015 Jun;27(6):644-8. doi: 10.1097/MEG.0000000000000353.
Classical features of autoimmune hepatitis (AIH) may be altered during the abrupt onset of the disease. Corticosteroid therapy can be life-saving, but its use in the fulminant presentation of AIH (F-AIH) remains controversial. We aimed to assess the clinical features of patients with F-AIH and to describe the role of corticosteroids in this population.
We retrospectively analyzed 154 adult patients with fulminant hepatic failure who were admitted to six liver transplantation (LT) programs. The AIH simplified criteria were used to identify patients with F-AIH.
We identified 40 (26%) patients with F-AIH. Compared with other etiologies, patients with F-AIH presented a longer interval from jaundice to encephalopathy (26 vs. 16 days, P=0.02) and a lower Model for End-Stage Liver Disease (MELD) score on admission (29 vs. 33, P=0.002). Overall, 25 (62%) patients with F-AIH underwent LT, eight (20%) patients survived, and seven (18%) died without LT. Seventeen patients received corticosteroids therapy, of whom seven (41%) survived without LT. Among the treated patients, higher MELD score and encephalopathy grade of 3 or more were associated significantly with corticosteroid failure.
Patients with F-AIH have a more indolent presentation compared with the non-F-AIH population. Altogether, only eight (20%) patients presenting with F-AIH survived without LT. A subset of patients with F-AIH and an initial MELD score less than 27 and low-grade hepatic encephalopathy might benefit from administration of corticosteroids.
自身免疫性肝炎（AIH）的典型特征在疾病突然发作时可能会发生改变。皮质类固醇疗法可能挽救生命，但其在暴发性AIH（F-AIH）中的应用仍存在争议。我们旨在评估F-AIH患者的临床特征，并描述皮质类固醇在该人群中的作用。
我们回顾性分析了154例入住六个肝移植（LT）项目的暴发性肝衰竭成年患者。采用AIH简化标准来识别F-AIH患者。
我们识别出40例（26%）F-AIH患者。与其他病因相比，F-AIH患者从黄疸到肝性脑病的间隔时间更长（26天对16天，P=0.02），入院时终末期肝病模型（MELD）评分更低（29对33，P=0.002）。总体而言，25例（62%）F-AIH患者接受了肝移植，8例（20%）患者存活，7例（18%）未接受肝移植死亡。17例患者接受了皮质类固醇治疗，其中7例（41%）未接受肝移植存活。在接受治疗的患者中，较高的MELD评分和3级或以上的肝性脑病与皮质类固醇治疗失败显著相关。
与非F-AIH人群相比，F-AIH患者的临床表现更为隐匿。总的来说，只有8例（20%）F-AIH患者未接受肝移植存活。一部分初始MELD评分低于27且肝性脑病程度较低的F-AIH患者可能从皮质类固醇治疗中获益。
