Controversies in Primary Sclerosing Cholangitis

• MRCP should be used as the initial investigation to diagnose PSC as it has similar accuracy without the invasiveness of ERCP. • Patients with a high clinical suspicion of PSC but with normal cholangiogram should undergo liver biopsy to exclude small duct PSC. Likewise, liver biopsy is indicated if an overlap with autoimmune hepatitis is suspected. In this case, treatment with corticosteroids or other immunosuppressants may be beneficial. • All patients diagnosed with PSC should have serum IgG4 measured, not only to help exclude IgG4-associated cholangitis, but also to identify a subgroup of patients at higher risk for a more aggressive course of PSC. • Use of ursodeoxycholic acid is not routinely recommended; use of high dose regimens (>25 mg/kg/day) is contraindicated due to increased risk of clinical deterioration. Drugs under investigation include antibiotics, supplements such as docosahexaenoic acid, fibrates, nor-UDCA, retinoic acid and anti-fibrotics. • Individuals with PSC have increased risk for hepatobiliary and colorectal malignancies. Use of surveillance strategies is highly recommended.