World J Gastroenterol. 2012 Jan 7;18(1):1-15. doi: 10.3748/wjg.v18.i1.1.
Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to fibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effective medical therapy is available. Liver transplantation (LTX) is the only curative treatment and at present PSC is the main indication for LTX in the Scandinavian countries. Close to half of the PSC patients experience one or more episodes of acute cellular rejection (ACR) following transplantation and approximately 1/5 of the transplanted patients develop recurrent disease in the graft. In addition, some reports indicate that ACR early after LTX for PSC can influence the risk for recurrent disease. For these important post-transplantation entities affecting PSC patients, we have reviewed the current literature on epidemiology, pathogenesis, treatment and the possible influence of rejection on the risk of recurrent disease in the allograft.
原发性硬化性胆管炎（PSC）是一种慢性进行性炎症性疾病，影响胆管，导致大多数患者发生纤维化，最终发展为肝硬化。其病因不明，到目前为止尚无有效的药物治疗方法。肝移植（LTX）是唯一的治愈性治疗方法，目前 PSC 是斯堪的纳维亚国家 LTX 的主要适应证。接近一半的 PSC 患者在移植后经历一次或多次急性细胞排斥反应（ACR），大约五分之一的移植患者在移植物中发生复发性疾病。此外，一些报告表明，LTX 后早期的 ACR 可能会影响复发性疾病的风险。对于这些影响 PSC 患者的重要移植后实体，我们回顾了关于流行病学、发病机制、治疗以及排斥反应对移植物复发性疾病风险影响的现有文献。
