Prognosis

Chronic inflammatory processes of the bile ducts result in progressive hepatic fibrosis, and ultimately cirrhosis and end-stage liver disease for most patients.

Natural history

Survival is significantly less than that of age- and sex-matched control populations.[6]​[47]​​[94] Life expectancy is reduced primarily by death from liver failure and hepatobiliary malignancies (cholangiocarcinoma in particular). The mean survival from the time of diagnosis to death or liver transplantation is 10-22 years.[2]

Stage of the disease at the time of diagnosis, the rate of disease progression, and the development of cholangiocarcinoma influence survival in the individual patient. Patients with symptoms at diagnosis generally have a worse prognosis (shorter expected survival) than asymptomatic patients.[26][94][95] Asymptomatic patients typically develop symptoms within several years of diagnosis.

Liver transplantation improves survival in patients with advanced liver disease due to PSC, with a 10-year post-transplant survival of 70%. Of patients with PSC, 13% to 35% eventually undergo liver transplantation.[4][6] Cholangiocarcinoma is often diagnosed at advanced, incurable stages due to the inability to identify patients at increased risk for the development of cholangiocarcinoma and the inability to identify early markers of malignancy. By this time, liver transplantation is usually contraindicated due to the risk of tumour recurrence and poor survival.

There is no correlation between the severity of inflammatory bowel disease symptoms and the course of PSC. Additionally, treatment of inflammatory bowel disease, including colectomy, does not alter the progression of PSC.[96]

Patients with small-duct PSC tend to have a more indolent course with better survival and less risk of cholangiocarcinoma than patients with classic, large-duct disease.[97][98] However, small-duct PSC remains a progressive disease that can also lead to end-stage liver disease. Patients with small-duct PSC can progress to large-duct PSC over time.

The presence of overlapping autoimmune hepatitis does not appear to change the course of PSC. Although transplant-free survival in patients with PSC-autoimmune hepatitis overlap syndrome appears to be higher than in PSC alone,[50][99] survival is worse than for those with autoimmune hepatitis alone.[100] Corticosteroid therapy leads to a significant reduction in aminotransferase levels,[50] although it is unclear whether corticosteroids alter the natural history of PSC in these patients. Patients with PSC-autoimmune hepatitis overlap syndrome are often initially diagnosed as having purely autoimmune hepatitis (based on the diagnostic criteria of the International Autoimmune Hepatitis Group[50]) and become resistant to immunosuppression. Subsequent cholangiography (after the initial diagnosis) then demonstrates changes typical of PSC.[7]

Post-liver transplant PSC recurrence

Recurrence in the graft after liver transplantation occurs in 10% to 20% of patients and can lead to graft failure requiring re-transplantation.[3][72][73][74][75] Recurrent PSC can be diagnosed more than 90 days post liver transplantation based on progressive biliary strictures seen on cholangiography or histology findings, provided other identifiable causes have been ruled out (e.g., allograft rejection).[2][3]

Prognostic models

Age, bilirubin, and histological stage are the most consistently identified independent predictors of survival.[94][101] Mathematical models based on such factors have been developed to predict survival for a particular patient. The revised Mayo risk score, which is the most widely used model, uses easily obtainable clinical and biochemical information (age, serum bilirubin level, serum alanine aminotransferase, serum albumin, and history of variceal bleeding) and does not require a liver biopsy.[102] Mayo Clinic: revised natural history model for primary sclerosing cholangitis Opens in new window

The main limitation of all prognostic models in PSC is the inability to predict the development of cholangiocarcinoma.

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