Immunoglobulin G4-related sclerosing cholangitis in a 3 years of age boy

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IgG4-RD). IgG4-RD mostly has been described in adults; hence, it is a relatively new disease and generally unknown to pediatricians. There are few reported cases about pediatric IgG4-RD. This report describes a 3 years of age boy who presented with abnormal liver function test, irregular dilatation of hepatic duct, and intermittent blood in stool which are initially diagnosed with autoimmune sclerosing cholangitis and ulcerative colitis. During follow-up, the patient was found to have an elevated IgG4 level. The liver biopsy showed numerous IgG4 positive plasmocytic cells infiltrating (>10/high power field). It fulfills the criteria of IgG4-SC. He was finally diagnosed as IgG4-SC. Within 2 years treatment, he had good response with immunosuppressant therapy.