Purpose of review: Identify advances in diagnosis, treatment, and understanding of autoimmune hepatitis.
Recent findings: Autoimmune hepatitis can present as an acute or fulminant hepatitis in the absence of pre-existent fibrotic disease. Celiac disease can resemble it, and treatment must include corticosteroids. Centrilobular necrosis is within the histologic spectrum of autoimmune hepatitis, and variant syndromes may be refractory to conventional therapies. Discriminative indices developed by head-to-head comparisons between the classical disorders may be useful in identifying true variants, and antibodies to chromatin may have prognostic value in predicting relapse after drug withdrawal. Multiple drugs and infectious agents can trigger the disease, and cytokine imbalances are important pathogenic factors. Recurrent or de novo autoimmune hepatitis must be considered as a cause of graft dysfunction after liver transplantation. Pregnancy is generally well tolerated, and it may attenuate the disease. After transplantation, pregnancy should be deferred until after the second year.
Summary: Autoimmune hepatitis has diverse presentations and manifestations. It must be considered in all patients with acute and chronic liver disease and in all cases of allograft dysfunction after liver transplantation.