Pediatric Autoimmune Liver Diseases: Autoimmune Hepatitis and Primary Sclerosing Cholangitis

Abstract

In chronic hepatitis, a broad differential diagnosis should be considered to accurately identify the cause(s) of liver injury. Autoimmune liver diseases (autoimmune hepatitis, primary sclerosing cholangitis, overlap syndrome) can occur in the setting of limited symptoms; therefore, a high index of suspicion and appropriate diagnostic workup should be performed. Most children with autoimmune hepatitis achieve sustained remission with medical therapy; however, there are no equivalent therapies for primary sclerosing cholangitis that impact the progression of disease. Research should include biomarker studies to predict histologic remission in autoimmune hepatitis and mechanistic studies to define future treatment targets for primary sclerosing cholangitis.

Keywords: Autoimmune hepatitis; Chronic hepatitis; Overlap syndrome; Pediatric liver transplantation; Primary sclerosing cholangitis.