Although several studies describe the natural history of primary biliary cirrhosis, many have been reported from tertiary referral centers.
In this study, a research team from England examined the prognosis of primary biliary cirrhosis in a comprehensive geographically defined cohort.
They followed up 770 primary biliary cirrhosis patients, from 1987 to 1994, until either death, transplantation, or censor on January 1, 2000.
The team used interview, review of case notes, and death certificates to obtain their data.
The survival data was analyzed using Kaplan-Meier methods and Cox regression.
|26% of patients developed liver failure by 10 years after diagnosis.|
The researchers found median patient survival to be 9 years from diagnosis.
They identified the most common symptoms at diagnosis to be pruritus (19%) and fatigue (21%).
Patient age, alkaline phosphatase, albumin, and bilirubin at diagnosis independently predicted survival in Cox modeling. However, prothrombin time and histologic stage did not independently affect survival.
It was calculated that observed survival was predicted well by this model, and by the Mayo prognostic score (R2M = 0.37 and 0.18, respectively).
R2M is a likelihood-based measure of the percentage information gain from the model due to covariates.
The research team found that 26% of patients had developed liver failure by 10 years after diagnosis.
During the study period, 42% of patient deaths were caused by liver disease.
In addition, 39 patients had liver transplantations by the censor date.
The team found survival to be much poorer in the study patients, than for an age- and sex-matched control population.
Dr Martin Prince's team concluded, "Although primary biliary cirrhosis is often now diagnosed at an early stage, the diagnosis still carries important prognostic implications".
"A significant proportion of patients develop liver failure, require transplantation, or die prematurely after this diagnosis".
In a related editorial, in the same publication, Dr Marshall Kaplan also discusses the diagnosis of primary biliary cirrhosis, as well as current and future treatments.