Autoimmune hepatitis.

Abstract

Autoimmune hepatitis (AIH) is a progressive inflammatory hepatitis of unknown etiology that is responsive to immunosuppressive therapy. The diagnosis of AIH should be guided by the descriptive criteria and scoring system set forth by the International Autoimmune Hepatitis Group. Standard therapy is prednisone with or without azathioprine (AZA). Combination therapy with prednisone and AZA is preferred, as it allows treatment with lower individual doses of each drug and is thus associated with fewer side effects. Treatment goals include complete biochemical, clinical, and histologic remission. Treatment outcomes include complete remission with or without relapse, incomplete response, or treatment failure. Treatment withdrawal, once remission has been attained, may be associated with relapse of disease. Recurrent relapse may be addressed with long-term, lower dose maintenance treatment with prednisone or AZA. Incomplete response is addressed by attempting high-dose immunosuppressive regimens or by considering investigational medical regimens. Treatment failure is also addressed by considering investigational medical regimens. Medication toxicity is addressed with dose reduction or drug discontinuation. Should the patient progress to decompensated cirrhosis, liver transplantation is an effective treatment for AIH. Calcium and vitamin D supplementation, a well-balanced diet, and exercise are advocated in patients with AIH. Cyclosporine, mycophenolate mofetil, and tacrolimus are promising agents among the new therapies for autoimmune hepatitis. Unfortunately, newer therapeutic agents have been studied in small numbers. Larger, controlled treatment trials are needed to expand the repertoire of therapeutics to treat patients with fewer side effects and to provide alternatives for patients who are refractory to conventional therapy.