Balan V, LaRusso N F
Mayo Clinic, Rochester, Minnesota, USA.
Gastroenterol Clin North Am. 1995 Sep;24(3):647-69.
PSC is the most common and most important hepatobiliary disease seen in association with IBD. Approximately 5% of all patients with CUC have PSC, and most patients with PSC ultimately develop IBD, usually CUC. PSC and CUC appear to be associated diseases-one does not cause the other, but common pathogenic mechanisms are likely involved. PSC alone does not differ from PSC with IBD with regard to clinical, biochemical, cholangiographic, and hepatic histologic features. There is an overlap syndrome of CAH and PSC in patients with CUC suggesting that patients with CAH and CUC should have a cholangiogram. Colectomy in patients with PSC and CUC does not influence the PSC and, if done for colitic indications, should be accompanied by an ileal pouch-anal anastomosis. Serologic markers are being identified, which are frequently found in PSC with or without CUC, including markers for the dreaded complication of cholangiocarcinoma. Unfortunately, patients with PSC and CUC are doubly at risk for malignancies of the colon and biliary system. Medical therapies are being assessed that may beneficially affect both PSC and CUC, and liver transplantation is life-saving for patients with advanced PSC. Although CAH and gallstones are also found in association with IBD, they are much less common and of considerably less clinical importance than PSC associated with IBD.
原发性硬化性胆管炎（PSC）是与炎症性肠病（IBD）相关的最常见且最重要的肝胆疾病。所有溃疡性结肠炎（CUC）患者中约5%患有PSC，而大多数PSC患者最终会发展为IBD，通常是CUC。PSC和CUC似乎是相关疾病——二者并非因果关系，但可能涉及共同的致病机制。就临床、生化、胆管造影和肝脏组织学特征而言，单纯的PSC与合并IBD的PSC并无差异。CUC患者存在慢性活动性肝炎（CAH）和PSC的重叠综合征，这表明患有CAH和CUC的患者应进行胆管造影。PSC和CUC患者行结肠切除术不会影响PSC，若因结肠炎指征进行结肠切除术，则应同时行回肠储袋肛管吻合术。正在确定血清学标志物，其在合并或不合并CUC的PSC中均常见，包括胆管癌这一可怕并发症的标志物。不幸的是，PSC和CUC患者患结肠癌和胆道系统恶性肿瘤的风险加倍。正在评估可能对PSC和CUC均产生有益影响的药物治疗方法，肝移植对晚期PSC患者可挽救生命。虽然CAH和胆结石也与IBD相关，但它们比与IBD相关的PSC少见得多，临床重要性也低得多。
