自身免疫性肝病重叠综合征的诊断和治疗

Diagnosis and Management of Overlap Syndrome of Autoimmune Liver Disease

自身免疫性肝病（AILD）是一组以肝脏病理损害和肝功能异常为主要表现的自身免疫性疾病，可分为自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）和原发性硬化性胆管炎（PSC），重叠综合征指同时具有其中两种疾病的临床和病理表现。重叠综合征相对少见，主要包括AIH—PBC和AIH—PSC。由于重叠综合征在临床表现、血清学和组织学方面综合了两种AILD的特点，其诊断和治疗有一定难度并存在争议。对其临床表现以及诊断和治疗方案进行深入研究有助于对该病的认识和防治。

Autoimmune liver disease （AILD） is a group of autoimmune disorders characterized by pathologic liver damage and liver dysfunction. Autoimmune hepatitis （AIH）, primary biliary cirrhosis （PBC）, and primary sclerosing cholangitis （PSC） are the three major types of AILD. Overlap syndrome is a relatively infrequent disease that has the combined clinical and pathological features of two major AILDs. It mainly consists of AIH-PBC and AIH-PSC overlap syndrome. As overlap syndrome shows the characteristics of two AILDs clinically and in serology and histology, the diagnosis and management are not yet straightforward and remain controversial. Therefore, a better understanding of the clinical phenotypes, diagnosis and treatment of AILD overlap syndrome may increase the knowledge and ability for its management.