Farrant J M, Hayllar K M, Wilkinson M L, Karani J, Portmann B C, Westaby D, Williams R
Department of Radiology, King's College Hospital, Denmark Hill, London, England.
Gastroenterology. 1991 Jun;100(6):1710-7. doi: 10.1016/0016-5085(91)90673-9.
The clinical features at the time of presentation and the outcome in 126 patients with primary sclerosing cholangitis were studied to clarify the natural history and prognosis in symptomatic and asymptomatic individuals. The median age of the patients at the time of presentation was 36 years, 62% were male, and 16% were asymptomatic. The median follow-up from time of presentation was 5.8 years. There were more patients who had liver transplants (21%) than patients who died of liver-related disease (16%); the estimated median survival to these end points was 12 years. Cholangiocarcinoma was found in 8 patients and in 23% of those undergoing liver transplantation. Asymptomatic patients had milder disease than symptomatic patients, but in a univariate analysis the presence of symptoms was not prognostically significant. On multivariate analysis, the following independent prognostic factors were found: hepatomegaly, splenomegaly, serum alkaline phosphatase, histological stage, and age. These features were combined to produce a prognostic model that should be valuable in the stratification of patients in clinical trials and in the timing of liver transplantation, particularly in those patients seen soon after presentation.
对126例原发性硬化性胆管炎患者的就诊时临床特征及预后进行了研究，以明确有症状和无症状个体的自然病程及预后。患者就诊时的中位年龄为36岁，62%为男性，16%无症状。自就诊起的中位随访时间为5.8年。接受肝移植的患者（21%）多于死于肝脏相关疾病的患者（16%）；至这些终点的估计中位生存期为12年。8例患者发现胆管癌，占接受肝移植患者的23%。无症状患者的病情比有症状患者轻，但单因素分析显示症状的存在在预后方面无显著意义。多因素分析发现以下独立预后因素：肝肿大、脾肿大、血清碱性磷酸酶、组织学分期和年龄。将这些特征结合起来建立了一个预后模型，该模型在临床试验中对患者分层以及肝移植时机的选择，尤其是对就诊后不久就被诊治的患者，应具有重要价值。
