Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis

被引:610
作者
Broome, U
Olsson, R
Loof, L
Bodemar, G
Hultcrantz, R
Danielsson, A
Prytz, H
SandbergGertzen, H
Wallerstedt, S
Lindberg, G
机构
[1] KAROLINSKA INST,HUDDINGE HOSP,DEPT MED,S-10401 STOCKHOLM,SWEDEN
[2] KAROLINSKA HOSP,DEPT MED,S-10401 STOCKHOLM,SWEDEN
[3] SAHLGRENS UNIV HOSP,DEPT MED,GOTHENBURG,SWEDEN
[4] ACAD HOSP,DEPT MED,UPPSALA,SWEDEN
[5] UNIV HOSP,LINKOPING,SWEDEN
[6] UNIV HOSP,UMEA,SWEDEN
[7] UNIV HOSP,LUND,SWEDEN
[8] REG HOSP,OREBRO,SWEDEN
[9] OSTA HOSP,GOTHENBURG,SWEDEN
关键词
ulcerative colitis; prognostic models; liver transplantation; cholangiocarcinoma;
D O I
10.1136/gut.38.4.610
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Background/Aims-The course primary sclerosing cholangitis (PSC) is highly variable and unpredictable. This study describes the natural history and outcome of PSC. These data were used to construct a prognostic model for patients with PSC. Methods-A total of 305 Swedish patients with PSC were studied. The median follow up time was 63 (1-194) months and all patients could be traced for follow up. Some 79 patients died or had a liver transplant. The prognostic significance of clinical, biochemical, and histological findings at the time of diagnosis were evaluated using multivariate analysis. Results-The estimated median survival from time of diagnosis to death or liver transplantation was 12 years. Cholangiocarcinoma was found in 24 (8%) of the patients and 134 (44%) of the patients were asymptomatic at the time of diagnosis. The estimated survival rate was significantly higher in the asymptomatic group (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.
引用
收藏
页码:610 / 615
页数:6
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