Lichtenstein David R
Section of Gastroenterology, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA.
Curr Gastroenterol Rep. 2011 Oct;13(5):495-505. doi: 10.1007/s11894-011-0213-9.
Several hepatobiliary abnormalities have been described in association with inflammatory bowel disease (IBD), including primary sclerosing cholangitis (PSC), small duct PSC, chronic hepatitis, cryptogenic cirrhosis, cholangiocarcinoma, and cholelithiasis. PSC is the most common biliary condition in patients with IBD, with an incidence ranging from 2.5% to 7.5%. PSC usually progresses insidiously and eventually leads to cirrhosis independent of inflammatory bowel disease activity. There is a very high incidence of cholangiocarcinoma and an elevated risk for developing colon cancer in patients with PSC. Medical therapy has not proven successful in slowing disease progression or prolonging survival. Treatment of symptoms due to cholestasis, such as pruritis and steatorrhea, is an important aspect of the medical care of patients with PSC. Our preferred treatment of pruritis due to cholestasis is with bile acid binding exchange resins, such as cholestyramine or colestipol. Endoscopic manipulation is recommended for treating complications of recurrent cholangitis or worsening jaundice in the setting of a dominant stricture, but endoscopic approaches have not been conclusively demonstrated to improve survival or decrease the need for liver transplantation. Liver transplantation remains the only effective treatment of advanced PSC, and should be considered in patients with complications of cirrhosis or intractable pruritis or fatigue.
多种肝胆异常已被描述与炎症性肠病（IBD）相关，包括原发性硬化性胆管炎（PSC）、小胆管PSC、慢性肝炎、隐源性肝硬化、胆管癌和胆石症。PSC是IBD患者中最常见的胆道疾病，发病率在2.5%至7.5%之间。PSC通常隐匿进展，最终导致肝硬化，与炎症性肠病活动无关。PSC患者胆管癌发病率非常高，患结肠癌的风险也增加。药物治疗在减缓疾病进展或延长生存期方面尚未被证明有效。治疗胆汁淤积引起的症状，如瘙痒和脂肪泻，是PSC患者医疗护理的一个重要方面。我们治疗胆汁淤积引起的瘙痒的首选药物是胆汁酸结合交换树脂，如考来烯胺或考来替泊。对于复发性胆管炎的并发症或在存在主导狭窄的情况下黄疸加重的情况，建议进行内镜操作，但内镜方法尚未被确凿证明能提高生存率或减少肝移植需求。肝移植仍然是晚期PSC的唯一有效治疗方法，对于有肝硬化并发症、顽固性瘙痒或疲劳的患者应考虑进行肝移植。
