Autoimmune hepatitis in children: diagnosis, pathology and treatment

Abstract

Autoimmune hepatitis (AIH) is characterized by progressive inflammation of the liver and destruction of liver parenchyma. Rare in absolute terms, it is nevertheless an important cause of noninfectious chronic liver disease in children. In many ways, the diagnosis and treatment of children with AIH has changed little over the last 10 years. However, in recent years, steady progress in defining the genetic, immunologic and potential environmental triggers that underlie this disease, in addition to increasing experience with a wider array of therapeutic agents, promises to expand our understanding and ability to treat AIH effectively. This review will summarize the current clinical and pathophysiological understanding of AIH in children, along with therapeutic options.

Keywords::

• autoantibodies
• azathioprine
• budesonide
• chloroquine
• cyclophosphamide
• cyclosporine
• deflazacort
• de novo autoimmune hepatitis
• liver pathology
• liver transplantation
• methotrexate
• mycophenolate mofetil
• mycophenolic acid
• polyendocrinopathy
• prednisone
• rapamycin
• scoring system
• tacrolimus
• ursodeoxycholic acid

Acknowledgements

This work was supported by NIH grant DK 07762