Autoimmune hepatitis (AIH) is a chronic autoimmune inflammatory liver disease with a sinister prognosis if untreated. Still, in patients on immunosuppressive treatment, there is increased morbidity and mortality, especially in nonresponders to standard therapy and difficult-to-manage AIH patients. Standard therapy includes prednisolone and azathioprine, while patients with azathioprine intolerance are offered mycophenolate mofetil. Nonresponders may be treated with other immunosuppressive drugs, for example, tacrolimus, ciclosporin, and anti-Tumor Necrosis Factor-α antibodies. Recently also rituximab has been introduced for AIH treatment. However, the evidence for the efficacy of these drugs is primarily based on treatment cohorts and case series from tertiary referral centers. In the present review, we provide the current knowledge regarding standard therapy and suggestions based on the literature for AIH patients being nonresponders to standard therapy and difficult-to-manage AIH patients to standard therapy. Drugs used as second- and third-line therapies are discussed with a suggested algorithm for treatment decisions.
Comprehensive Guide to Hepatitis Advances 2023, Pages 429-441,