Objective: To describe a natural history model for primary sclerosing chola
ngitis (PSC) that is based on routine clinical findings and test results an
d eliminates the need for liver biopsy.
Patients and Methods: Using the Cox proportional hazards analysis, we creat
ed a survival model based on 405 patients with PSC from 5 clinical centers.
Independent validation of the model was undertaken by applying it to 124 p
atients who were not included in the model creation.
Results: Based on the multivariate analysis of 405 patients, a risk score w
as defined by the following formula: R = 0.03 (age [y]) + 0.54 log(e) (bili
rubin [mg/dL]) + 0.54 log(e) (aspartate aminotransferase [U/L]) + 1.24 (var
iceal bleeding [0/1]) - 0.84 (albumin [g/dL]), The risk score was used to o
btain survival estimates up to 4 years of follow-up. Application of this mo
del to an Independent group of 124 patients showed good correlation between
estimated and actual survival,
Conclusions: A new model to estimate patient survival in PSC includes more
reproducible variables (age, bilirubin, albumin, aspartate aminotransferase
, and history of variceal bleeding), has accuracy comparable to previous mo
dels, and obviates the need for a liver biopsy.