原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征的诊治

Diagnosis and treatment of primary biliary cholangitis-autoimmune hepatitis overlap syndrome

自身免疫性肝病(AILD)包括原发性胆汁性胆管炎(PBC)、自身免疫性肝炎(AIH)、原发性硬化性胆管炎(PSC)。患者可在初诊时或随访的过程中出现2种AILD的特征,通常将这种情况称为“重叠综合征”,其中以PBC重叠AIH最为常见。与单纯PBC或AIH相比,PBC-AIH重叠综合征门静脉高压、消化道出血、腹水、死亡及肝移植发生率明显升高,病情进展也更迅速,因此,其早诊早治显得尤为重要。对近年PBC-AIH重叠综合征的诊治进展进行综述。

Autoimmune liver diseases(AILDs)include primary biliary cholangitis(PBC),autoimmune hepatitis(AIH),and primary sclerosing cholangitis.Some patients may have the features of two AILDs at initial diagnosis or during follow-up,which is usually referred to as“overlap syndrome”,among which PBC-AIH overlap is the most common type.Compared with those with PBC or AIH alone,the patients with PBC-AIH overlap syndrome tend to have higher incidence rates of portal hypertension,gastrointestinal bleeding,ascites,death,and liver transplantation,as well as more rapid disease progression,and therefore,early diagnosis and treatment is of great importance.This article reviews the recent advances in the diagnosis and treatment of PBC-AIH overlap syndrome.