Management of paediatric patients with autoimmune hepatitis in the light of current international guidelines. Literature review

Autoimmune hepatitis is a chronic immune‑mediated liver disease characterized by elevated serum IgG and specific circulating autoantibodies in the serum with typical histological features, which, without appropriate treatment, can progress to fibrosis, cirrhosis, and liver failure. Juvenile autoimmune hepatitis is usually a more aggressive disease than in adults and has its own characteristics. Autoimmune hepatitis in children is characterized by higher immunological activity: higher IgG levels. A higher incidence of positive antinuclear antibodies (ANA) and/or antismooth muscle antibodies (ASMA) is characteristic of autoimmune hepatitis type 1, while the presence of hepatorenal microsomal antibodies (anti‑LKM‑1) and/or antibodies to liver cytosol type 1 (anti‑LC‑1) is characteristic of autoimmune hepatitis type 2. Autoimmune hepatitis type 2 is considered a purely pediatric phenomenon, has a more severe course and more often requires intensive immunosuppressive therapy. Drug therapy for autoimmune hepatitis in children is divided into standard (corticosteroids and azathioprine) and alternative (mycophenolate mofetil, cyclosporine, tacrolimus, biological therapy with monoclonal antibodies). According to the latest international guidelines for the management of pediatric patients with autoimmune hepatitis from the European Association for the Study of the Liver (EASL), the European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the American Association for the Study of Liver Diseases (AASLD), all patients with autoimmune hepatitis are recommended to undergo liver biopsy before diagnosing and discontinuation of therapy. Immunosuppressive therapy itself should last at least three years. With timely diagnosis and appropriate therapy, the prognosis of the disease is favorable in most cases. Treatment failure is observed in approximately 10% of pediatric patients, who sometimes require liver transplantation.