Bunchorntavakul Chalermrat, Reddy K Rajender
Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, 2 Dulles, 3400 Spruce Street, Philadelphia, PA 19104, USA; Division of Gastroenterology and Hepatology, Department of Medicine, Rajavithi Hospital, College of Medicine, Rangsit University, Rajavithi Road, Ratchathewi, Bangkok 10400, Thailand.
Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, 2 Dulles, 3400 Spruce Street, Philadelphia, PA 19104, USA.
Clin Liver Dis. 2015 Feb;19(1):81-97. doi: 10.1016/j.cld.2014.09.005. Epub 2014 Nov 21.
Overlapping features between autoimmune hepatitis (AIH) and cholestatic disorders (primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), or indeterminate cholestasis), so-called overlap syndromes, usually have a progressive course toward cirrhosis and liver failure without adequate treatment. The diagnosis of overlap syndrome requires the prominent features of classic AIH and secondary objective findings of PBC or PSC. Empiric treatment for patients with AIH-PBC overlap is immunosuppressive therapy plus ursodeoxycholic acid. Empiric treatment for patients with AIH-PSC and AIH-cholestatic overlap is immunosuppressive therapy with or without ursodeoxycholic acid. Liver transplantation is indicated for patients who have end-stage liver disease.
自身免疫性肝炎(AIH)与胆汁淤积性疾病(原发性胆汁性肝硬化(PBC)、原发性硬化性胆管炎(PSC)或不明原因胆汁淤积)之间存在重叠特征,即所谓的重叠综合征,若未得到充分治疗,通常会朝着肝硬化和肝衰竭的方向进展。重叠综合征的诊断需要具备典型AIH的突出特征以及PBC或PSC的次要客观表现。AIH-PBC重叠患者的经验性治疗是免疫抑制疗法加熊去氧胆酸。AIH-PSC和AIH-胆汁淤积重叠患者的经验性治疗是免疫抑制疗法,可加用或不加用熊去氧胆酸。对于终末期肝病患者,需进行肝移植。