Franco J, Saeian K
Medical College of Wisconsin, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, USA.
Curr Gastroenterol Rep. 1999 Apr;1(2):95-101. doi: 10.1007/s11894-996-0006-8.
Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is the only option for patients with end-stage liver disease and yields excellent long-term survival in both groups.
原发性硬化性胆管炎（PSC）和原发性胆汁性肝硬化（PBC）是慢性进行性胆汁淤积性疾病，常导致胆汁性肝硬化。这些疾病的确切发病机制尚不清楚，但可能基于免疫因素。并发症范围从疲劳、瘙痒到终末期肝病。PBC患者发生肝细胞癌的风险较低，而胆管癌在PSC中常见且预后不佳。虽然熊去氧胆酸可有效减缓PBC的进展，但PSC尚无有效的药物治疗方法。肝移植是终末期肝病患者的唯一选择，两组患者均可获得良好的长期生存率。
