Open challenges in the management of autoimmune hepatitis.

Autoimmune Hepatitis (AIH) is a rare autoimmune disease of the liver with many open questions as regards its aetiopathogenesis, natural history and clinical management. The classical picture of AIH is chronic hepatitis with fluctuating elevation of serum transaminases and Immunoglobulin G levels, the presence of circulating autoantibodies and typical histological features. However, atypical presentations do occur and are not well captured by current diagnostic scores, with important consequences in terms of missed diagnoses and delayed treatments. AIH is treated with corticosteroids and immunosuppressive drugs but up to 40% of patients do not achieve full biochemical response and are at risk of progressing to cirrhosis and liver failure. Moreover, standard therapies are associated by significant side-effects which may impair the quality of life of patients living with AIH. However, advances in the understanding of the underlying immunology of AIH is raising the prospect of novel therapies and optimisation of existing therapeutic approaches to reduce side-effect burdens and potentially restore immunological tolerance. In this review we outline the clinical characteristics, aetiopathogenesis and management of AIH and current challenges in the diagnosis and management of AIH and provide evidence underlying the evolution of diagnostic and clinical management protocols.