Second-line and Third-line therapy for Autoimmune Hepatitis A position statement from the European Reference Network on Hepatological Diseases and the International Autoimmune Hepatitis Group.
Autoimmune hepatitis in the majority of patients responds well to standard immunosuppressive therapy with steroids and azathioprine, and while untreated disease is usually fatal, patients responding well to therapy have an excellent prognosis. However, about 10-20% of patients present therapeutic challenges due to an insufficient response or intolerable side-effects to standard therapy, requiring dose-adaptation or switch of therapy. While there is a fairly good agreement on second-line treatment options, there is very wide variation in the indication and use of possible third-line therapies. The European Reference Network on Hepatological Diseases (ERN RARE-LIVER) and the International Autoimmune Hepatitis Group (IAIHG) here offer a treatment algorithm for both children and adults that should help to standardize treatment approaches, in order to improve patient care as well as to allow comparison of treatment results in scientific publications.