Schrumpf E, Abdelnoor M, Fausa O, Elgjo K, Jenssen E, Kolmannskog F
Medical Department, Rikshospitalet, Oslo, Norway.
J Hepatol. 1994 Dec;21(6):1061-6. doi: 10.1016/s0168-8278(05)80618-x.
The increasing use of liver transplantation and new treatment regimens requires an accurate estimate of the prognosis in primary sclerosing cholangitis. To clarify the natural history and prognosis of this disease, we studied the clinical features at the time of presentation and the outcome in 77 consecutive patients admitted to our hospital. The median age at diagnosis of primary sclerosing cholangitis was 32.5 years; 66% of the patients were male; 76 had concomitant inflammatory bowel disease and two had celiac disease. Thirty-four patients were classified as asymptomatic at diagnosis of primary sclerosing cholangitis. The mean follow-up time was 6.2 years; 25 patients have died or been transplanted. Cholangiocarcinoma has been diagnosed in 11 patients (14%). Female patients have a significantly poorer survival rate than male patients. The bilirubin level was found to be an independent risk factor for both mortality/transplantation, and for the occurrence of cholangiocarcinoma. Age at diagnosis of primary sclerosing cholangitis was an additional risk factor of death/transplantation. As bilirubin is an important prognostic factor for the development of both cholangiocarcinoma and death/transplantation, the construction of prognostic indices seems to be of limited value in the timing of transplantation of the individual patient.
肝移植和新治疗方案的使用日益增加，这就需要准确评估原发性硬化性胆管炎的预后。为了阐明这种疾病的自然病程和预后，我们研究了我院连续收治的77例患者的就诊时临床特征及转归。原发性硬化性胆管炎诊断时的中位年龄为32.5岁；66%的患者为男性；76例伴有炎症性肠病，2例患有乳糜泻。34例患者在原发性硬化性胆管炎诊断时被分类为无症状。平均随访时间为6.2年；25例患者死亡或接受了移植。11例患者（14%）被诊断为胆管癌。女性患者的生存率明显低于男性患者。胆红素水平被发现是死亡/移植以及胆管癌发生的独立危险因素。原发性硬化性胆管炎诊断时的年龄是死亡/移植的另一个危险因素。由于胆红素是胆管癌发生以及死亡/移植的重要预后因素，因此预后指数的构建对于个体患者移植时机的判断价值有限。
