临床特征及诊断世界各地原发性胆汁性肝硬化(PBC)的发病率不尽一致。现一致认为:临床上良性或进展缓慢的PBC 较迄今所明确诊断的更为常见,常规生化检查确诊的病例中约10%无症状,因而实际发病率可能更高。由于PBC 特异的M_2抗原具有特征性,故酶联免疫吸附法(ELISA)将取代繁琐的免疫荧光法,并能对更早期或无症状的病例作出诊断。早期的免疫抑制治疗可阻止PBC 的自然病程,故对ELISA 阳性而肝功能正常的无症状患者应进行诊断性肝活检。 Clinical Features and Diagnosis The incidence of primary biliary cirrhosis (PBC) in different parts of the world is not consistent. It is now agreed that clinically benign or slow-moving PBC is more common than previously diagnosed, and about 10% of cases confirmed by routine biochemical tests are asymptomatic and may actually have a higher incidence. Because PBC-specific M_2 antigen is characteristic, enzyme-linked immunosorbent assay (ELISA) will replace tedious immunofluorescence and make the diagnosis of earlier or asymptomatic cases. Early immunosuppressive therapy can prevent the natural course of PBC, so positive for the ELISA and asymptomatic patients with normal liver function should be diagnosed liver biopsy.