Historical aspects and current understanding of autoimmune hepatitis. When is liver transplantation indicated? (Review)

Abstract

Autoimmune hepatitis (AIH) can occur at any age and is more common in women. The disease is a manifestation of autoimmune predisposition caused in genetically susceptible people exposed to certain environmental factors. The pathogenetic mechanism of AIH is not yet fully understood, but it involves an aggressive cellular immune response. The pathogenesis and severity of AIH also depend on various cytokines. This disease is characterized by elevated levels of transaminases – aspartate aminotransferase (AST) and alanine aminotransferase (ALT). Liver histology plays a crucial role in confirming or supporting the clinical diagnosis of AIH. Diagnosis of AIH remains a challenge in clinical practice. AIH is one of the few liver diseases for which pharmacologic treatment has been shown to improve survival. Standard treatment is based on high-dose prednisone alone or prednisolone plus azathioprine. It leads to disease remission in 80%-90% of patients. Approximately 20% of patients do not respond to the standard steroid treatment and are treated with second-line immunosuppressive drugs: mycophenolate mofetil, budesonide, cyclosporine, tacrolimus, everolimus, and sirolimus. There have been reports on the use of infliximab and rituximab. In the natural course of AIH and resistance to therapy, there is a tendency for cirrhosis to develop and for the disease to progress to an end stage. These patients, as well as those diagnosed with fulminant liver failure, require liver transplantation.