Primary sclerosing cholangitis is a chronic cholestatic liver disease with varying severity and progression.
Dr Jens Tischendorf and colleagues from Germany described the natural history of primary sclerosing cholangitis patients.
The research team evaluated the prognostic significance of clinical, biochemical, and cholangiographic findings constructing a novel prognostic model.
The researchers studied a population of 273 primary sclerosing cholangitis patients with a median follow-up time of 76 months.
Survival curves were analyzed by the Kaplan-Meier method.
The research team assessed the prognostic significance of clinical, biochemical, and cholangiographic features recorded at the time of diagnosis.
The team used multivariate analysis using Cox proportional-hazards regression models.
|40% of patients underwent liver transplantation|
|American Journal of Gastroenterology|
The researchers estimated that the median survival from the time of diagnosis to death or time of liver transplantation was 10 years.
The team reported that 40% of patients underwent liver transplantation.
Hepatobiliary malignancies were found in 14% patients of the entire primary sclerosing cholangitis population.
The researchers found that age, low albumin, and a persistent bilirubin elevation longer than 3 months were independent risk factors correlating with poor prognosis.
Hepatomegaly, splenomegaly, and dominant bile duct stenosis were independent risk factors with poor prognosis.
In addition, the team noted that intra- and extrahepatic ductal changes at the time of diagnosis were independent risk factors correlating with poor prognosis.
The team used these factors to construct a new prognostic model.
Dr Tischendorf's team concludes, “A persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome.”
“A new prognostic model was developed to predict progression of primary sclerosing cholangitis, which may be useful in timing of liver transplantation.”