Primary Sclerosing Cholangitis, an extraintestinal manifestation of IBD

Primary Sclerosing Cholangitis (PSC) is a chronic, progressive, cholestatic liver disease that is associated with IBD in approximately two thirds of cases. The diagnosis is often made in asymptomatic patients when abnormal liver function tests are noted in routine follow-up. If symptomatic, patients may complain of fatigue, itching, fever, right upper quadrant pain, and weight loss.
The traditional gold standard diagnostic test has been endoscopic retrograde cholangiopancreatography (ERCP), but noninvasive magnetic resonance cholangiography (MRCP) is increasingly used. It has been suggested that ERCP should remain the confirmatory test, given its higher specificity, or that perhaps it should be reserved for patients in whom the diagnosis is uncertain after MRCP. If an MRCP is negative and the index of suspicion is low, liver biopsy, rather than ERCP, may be the favored approach. Typical features at liver biopsy are periductal inflammation and fibrosis, bile ductular proliferation, and portal tract inflammation.

Ursodeoxycholic acid (UDCA) has been extensively studied in the treatment of PSC and is widely prescribed for this indication.
Steroids have not been shown to benefi t PSC patients, and trials of immunosuppressants have not demonstrated consistent benefi ts, though small studies of cyclosporine and tacrolimus have suggested that further investigation is merited. Tight strictures in the biliary tree can be treated endoscopically with balloon dilation or biliary stents.
Cholangiocarcinoma and colonic carcinoma Cholangiocarcinoma will develop in approximately 10% to 30% of patients with PSC , but to date there are no reliable means of identifying those at particular risk.PSC patients are also at particularly high risk of colonic dysplasia and cancer, and it is recommended that those with UC undergo yearly surveillance colonoscopy.