Liver disease in pediatric inflammatory bowel disease

Abstract

Hepatobiliary diseases are among the most common extraintestinal manifestations of inflammatory bowel disease (IBD). These include immune-mediated liver diseases, namely, primary sclerosing cholangitis, autoimmune hepatitis, and an overlap condition between the two, termed autoimmune sclerosing cholangitis. More recently, an additional entity that likely shares an immunological basis, known as IgG4-associated cholangitis (IAC), has been defined and observed to also occur in association with IBD. A number of hepatobiliary manifestations can result from the adverse effects of medications used to treat pediatric IBD, such as methotrexate, thiopurines, and anti-tumor necrosis factor-α antibodies. Another category of hepatobiliary involvement is that which reflects the pathophysiology of IBD, such as cholelithiasis. Abnormal liver biochemistry is common in children with IBD. While often transient and of little consequence, such abnormalities may occasionally herald serious underlying liver disease or medication toxicity. The challenge lies in determining which patients warrant further investigation and intervention versus simple observation. This chapter strives to facilitate this task by providing an overview of and suggesting clinical approaches to various hepatobiliary conditions associated with pediatric IBD.