Indian National Association for Study of the Liver Guidance Document on Difficult to Treat Autoimmune Hepatitis

Autoimmune hepatitis (AIH) is an immune-mediated inflammatory disorder. It is a highly heterogeneous entity, having a wide range of presentations from asymptomatic chronic hepatitis to cirrhosis and acute liver failure. In consonance with the variabilities in presentation, there are also variations in response to treatment, depending on disease phenotype, presentation, extent of fibrosis, and the presence of comorbidities. In addition, pediatric AIH and AIH postliver transplant have their individual nuances. Addressing such areas to identify strategies for best practices is an unmet goal in this population of "difficult to treat" AIH. While established guidelines exist for AIH overall, specific guidance documents for phenotypes of difficult-to-treat AIH are lacking. The current document provides consensus-based guidance statements on definitions and criteria for determining difficult-to-treat AIH, encompassing the spectrum from acute AIH to AIH with compensated and decompensated cirrhosis, drug-induced autoimmune-like hepatitis, overlap syndromes, AIH in the presence of pregnancy, unique populations of pediatrics and postliver transplant AIH, and the impact of concomitant comorbidities.