Published online Jun 21, 2020. doi: 10.3748/wjg.v26.i23.3126
Peer-review started: December 23, 2019
First decision: March 23, 2020
Revised: April 7, 2020
Accepted: June 5, 2020
Article in press: June 5, 2020
Published online: June 21, 2020
Core tip: Elevated serum IgG4 titres and/or abundant IgG4-positive plasma cell infiltrates in the liver and bile ducts are present in up-to one-quarter of patients with primary sclerosing cholangitis (PSC). It should be distinguished from IgG4-related sclerosing cholangitis. PSC with high IgG4 have a different clinical phenotype to those with normal IgG4, with higher bilirubin and alkaline phosphatase, higher PSC Mayo Risk Score, more advanced liver disease, shorter time to liver transplantation, more aggressive colitis and shorter time to colectomy. Corticosteroids may provide biochemical improvement but rarely results in cholangiographic resolution. Concerns include steroid toxicity, infection and disease progression in those with advanced cirrhosis.