Pharmacological management of autoimmune hepatitis

Introduction: Without prompt immunosuppressive treatment, autoimmune hepatitis is a devastating, albeit rare, liver disease. It affects both adults and children, being particularly aggressive in the latter. Eighty per cent of patients respond satisfactorily to treatment; the other 20% progress to end-stage liver disease and require transplantation. Areas covered: This review emphasizes the importance of a timely diagnosis of autoimmune hepatitis and provides a practical guide for its treatment. The authors summarize the treatment options available for autoimmune hepatitis and stress that most patients respond successfully to standard treatment with prednisolone and azathioprine, two well-tried and inexpensive drugs. The authors also review the options for difficult-to-treat patients (non-responders and frequent relapsers), for whom newer immunosuppressive agents -- usually employed as anti-rejection drugs -- have been tried with variable success. Expert opinion: Autoimmune hepatitis is exquisitely responsive to immunosuppression but treatment must be started as soon as possible to achieve full remission and halt progression of liver disease. New strategies aiming at treating the unresponsive patients and at specifically curbing the liver autoimmune attack, without provoking unwanted systemic side effects, are being investigated and may be available within the next 5 years.