Talwalkar Jayant A, Lindor Keith D
Division of Gastroenterology and Hepatology, Mayo Clinic and Foundation, Rochester, MN 55905, USA.
Lancet. 2003 Jul 5;362(9377):53-61. doi: 10.1016/S0140-6736(03)13808-1.
Primary biliary cirrhosis is a chronic cholestatic liver disease of adults. This disorder is characterised histologically by chronic non-suppurative destruction of interlobular bile ducts leading to advanced fibrosis, cirrhosis, and liver failure. The precise aetiopathogenesis of primary biliary cirrhosis remains unknown, although dysregulation of the immune system and genetic susceptibility both seem to be important. Affected patients are typically middle-aged women with abnormal serum concentrations of alkaline phosphatase. Presence of antimitochondrial antibody in serum is almost diagnostic of the disorder. Identification of primary biliary cirrhosis is important, because effective treatment with ursodeoxycholic acid has been shown to halt disease progression and improve survival without need for liver transplantation. However, therapeutic options for disease-related complications-including fatigue and metabolic bone disease-remain unavailable. Mathematical models have been developed that accurately predict the natural history of primary biliary cirrhosis in individuals. Despite advances in understanding of the disease, it remains one of the major indications for liver transplantation worldwide.
原发性胆汁性肝硬化是一种成人慢性胆汁淤积性肝病。该疾病在组织学上的特征是小叶间胆管的慢性非化脓性破坏，导致进行性纤维化、肝硬化和肝衰竭。尽管免疫系统失调和遗传易感性似乎都很重要，但原发性胆汁性肝硬化的确切病因发病机制仍不清楚。受影响的患者通常为中年女性，血清碱性磷酸酶浓度异常。血清中抗线粒体抗体的存在几乎可诊断该疾病。原发性胆汁性肝硬化的诊断很重要，因为已证明熊去氧胆酸的有效治疗可阻止疾病进展并提高生存率，而无需进行肝移植。然而，针对包括疲劳和代谢性骨病在内的与疾病相关并发症的治疗选择仍然没有。已经开发出数学模型，可以准确预测个体原发性胆汁性肝硬化的自然病程。尽管对该疾病的认识有所进步，但它仍然是全球肝移植的主要指征之一。
