PSC is a chronic inflammatory and often progressive disorder of unknown cause, reported in up to 4% of IBD patients (in UC > CD).
Fibrosis and subsequently strictures develop in the intra- and extrahepatic biliary tree, mainly affecting medium and large bile ducts.
This can result in end-stage liver disease and associates with an increased risk of cholangiocarcinoma.
Patients with concurrent IBD have a significantly higher rate of colorectal carcinoma compared to UC patients without PSC.
Patients are often asymptomatic. Symptoms of PSC include intermittent malaise, pruritus, fever, chills, night sweats, and right upper abdominal quadrant pain.
Median survival without liver transplantation reported from tertiary referral centres (10-12 years) is lower than that of population based studies (>20 years).
The main complications are progressive biliary strictures, and cholangiocarcinoma. Rarer complications of PSC include: cholestasis, cholangitis, cholecystolithiasis, cholangiocarcinoma, colorectal carcinoma, osteoporosis, vitamin deficiency and steatorrhea. PSC may be associated with autoimmune diseases such as autoimmune thyroid disease, type 1 diabetes and celiac disease. An overlap syndrome with auto-immune hepatitis occurs in 10% of patients.
Liver transplantation is the only therapy that can cure PSC. Outcomes are favourable with 5-year survival rates close to 85%. PSC patients with end-stage liver disease should be assessed for liver transplantation according to standard guidelines. In addition, patients with intractable pruritus and selected cases with recurrent, severe bacterial cholangitis can be referred for liver transplantion. The selection for and timing of transplantation in PSC patients is difficult, due to the unpredictable disease course and the risk of cholangiocarcinoma.