文摘

背景/目的——课程原发性硬化性胆管炎(PSC)是高度可变和不可预测的。本研究描述了自然历史和PSC的结果。这些数据被用来构造一个PSC患者预后模型。方法——总共305瑞典PSC患者进行了研究。平均随访时间为63(1 - 194)个月,所有患者可以追踪跟进。大约79名病人死亡或接受了肝脏移植。的预后意义的临床、生化和组织学结果在诊断时使用多变量分析进行评估。诊断结果,估计平均生存时间的死亡或肝移植是12年。发现胆管癌24例(8%)患者和134例(44%)的患者无症状的诊断。估计生存率明显高于无症状组(p < 0.001)。 However, 29 (22%) of the asymptomatic patients became symptomatic during the study period. It was found that age, serum bilirubin concentration, and histological stage at the time of diagnosis were independent predictors of a bad prognosis. These variables were used to construct a prognostic model. CONCLUSIONS--This prognostic model developed from a large homogeneous population of PSC patients should be of value for the timing of transplantation and patient counselling in PSC.