Dabaghi R E, Lester R
Am Fam Physician. 1986 May;33(5):155-65.
Primary biliary cirrhosis often presents insidiously but progresses relentlessly in its later stages. Asymptomatic patients, however, may have a relatively benign course. Clinical, laboratory and histologic characteristics support an autoimmune pathogenesis. The complications of primary biliary cirrhosis are secondary to severe cholestasis, portal hypertension and progressive hepatocellular dysfunction. Currently, treatment is only supportive; no drug regimen has been shown to alter the disease course.
原发性胆汁性肝硬化通常起病隐匿,但在后期会持续进展。然而,无症状患者的病程可能相对良性。临床、实验室及组织学特征支持自身免疫性发病机制。原发性胆汁性肝硬化的并发症继发于严重胆汁淤积、门静脉高压和进行性肝细胞功能障碍。目前,治疗仅为支持性治疗;尚无药物疗法被证明能改变疾病进程。