Autoimmune hepatitis is a rare chronic inflammatory liver disease, affecting all ages, characterised by elevated transaminase and immunoglobulin G levels, positive autoantibodies, interface hepatitis at liver histology and good response to immunosuppressive treatment. If untreated, it has a poor prognosis. The aim of this review is to summarize the evidence for standard treatment and to provide a systematic review on alternative treatments for adults and children. Standard treatment is based on steroids and azathioprine, and leads to disease remission in 80%-90% of patients. Alternative first line treatment has been attempted with budesonide or cyclosporine, but their superiority compared to standard treatment remains to be demonstrated. Second-line treatments are needed for patients not responding or intolerant to standard treatment. No randomized controlled trials have been performed for second-line options. Mycophenolate mofetil is the most widely used second-line drug, and has good efficacy particularly for patients intolerant to azathioprine, but has the major disadvantage of being teratogenic. Only few and heterogeneous data on cyclosporine, tacrolimus, everolimus and sirolimus are available. More recently, experience wit...Continue Reading
Prednisone for chronic active liver disease: dose titration, standard dose, and combination with azathioprine compared
Maintenance of remission in autoimmune chronic active hepatitis with azathioprine after corticosteroid withdrawal
Controlled trial of a thymic hormone extract (Thymostimulin) in 'autoimmune' chronic active hepatitis
Late results of the Royal Free Hospital prospective controlled trial of prednisolone therapy in hepatitis B surface antigen negative chronic active hepatitis
Azathioprine versus prednisone in non-alcoholic chronic liver disease (CLD). Relation to a serological classification
Cyclosporine in the management of corticosteroid-resistant type I autoimmune chronic active hepatitis
The successful treatment of autoimmune hepatitis with 6-mercaptopurine after failure with azathioprine
Exacerbated autoimmune hepatitis successfully treated with leukocytapheresis and bilirubin adsorption therapy
Ursodeoxycholic acid as adjunctive therapy for problematic type 1 autoimmune hepatitis: a randomized placebo-controlled treatment trial
International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis
Mycophenolate mofetil for maintenance of remission in autoimmune hepatitis in patients resistant to or intolerant of azathioprine
Successful tacrolimus therapy for a severe recurrence of type 1 autoimmune hepatitis in a liver graft recipient
Thiopurine methyltransferase phenotype and genotype in relation to azathioprine therapy in autoimmune hepatitis
Azathioprine metabolite measurements in the treatment of autoimmune hepatitis in pediatric patients: a preliminary report
Pharmacokinetics and pharmacodynamic action of budesonide in early- and late-stage primary biliary cirrhosis
Effectiveness and safety of ciclosporin as therapy for autoimmune diseases of the liver in children and adolescents
Mycophenolate mofetil for the treatment of autoimmune hepatitis in patients refractory to standard therapy
Increased production of granulocyte-macrophage colony-stimulating factor in Crohn's disease--a possible target for infliximab treatment
Transplant immunosuppressive agents in non-transplant chronic autoimmune hepatitis: the Canadian association for the study of liver (CASL) experience with mycophenolate mofetil and tacrolimus
Empiric therapy of autoimmune hepatitis with mycophenolate mofetil: comparison with conventional treatment for refractory disease
Retrospective evaluation of cyclosporine in the treatment of presumed idiopathic chronic hepatitis in dogs
Comparison of mycophenolate mofetil with standard treatment for autoimmune hepatitis: a meta-analysis
Persistent cholestasis resulting from duodenal papillary carcinoma in an adolescent male: A case report
Efficacy and safety of chloroquine plus prednisone for the treatment of autoimmune hepatitis in a randomized trial
A comprehensive autoantigen-ome of autoimmune liver diseases identified from dermatan sulfate affinity enrichment of liver tissue proteins
Autoimmune Hepatitis-Immunologically Triggered Liver Pathogenesis-Diagnostic and Therapeutic Strategies
Azathioprine Monotherapy Is Equivalent to Dual Therapy in Maintaining Remission in Autoimmune Hepatitis.
Rescue of autoimmune hepatitis by soluble MHC class II molecules in an altered concanavalin A-induced experimental model
Reply to: "Both tacrolimus and mycophenylate mophetil should be considered second-line therapy for autoimmune hepatitis".
The Asian Pacific Association for the Study of the Liver clinical practice guidance: the diagnosis and management of patients with autoimmune hepatitis.
A case report on severe nivolumab-induced adverse events similar to primary sclerosing cholangitis refractory to immunosuppressive therapy.
Society for Immunotherapy of Cancer (SITC) clinical practice guideline on immune checkpoint inhibitor-related adverse events.
Quantitative multiparametric MRI as a non-invasive stratification tool in children and adolescents with autoimmune liver disease.
Clinical Trials Mentioned
Autoimmune hepatitis formerly called lupoid hepatitis, is a chronic, autoimmune disease of the liver that occurs when the body's immune system attacks liver cells causing the liver to be inflamed. Discover the latest research on autoimmune hepatitis here.
Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.