Recent updates on the management of autoimmune hepatitis

Abstract

Autoimmune hepatitis (AIH) is an immunoinflammatory chronic liver disease with dynamic and rather heterogeneous disease manifestations. A trend of increasing prevalence of AIH has been observed worldwide, along with a relative increase in the percentage of male patients. AIH is characterized and diagnosed based on serum biochemistry and liver histology: elevated aminotransferases and serum immunoglobulin G (IgG), the presence of serum anti-nuclear antibody or anti-smooth muscle antibody, and interface lympho-plasmacytic hepatitis. Clinical manifestations differ among disease subtypes with distinct time-frames, i.e., AIH with a chronic insidious onset, and acute-onset AIH (the diagnosis of which is often challenging due to the lack of typical serum findings). The absence of disease-specific biomarkers or histological findings may expand the disease phenotype into drug-induced AIH-like liver injury. Corticosteroids and azathioprine are recommended first-line treatments for AIH. The complete normalization of aminotransferases and serum IgG is an essential treatment response to ensure long-term overall survival. An incomplete response or intolerance to these drugs is considered an indication for second-line treatment, especially with mycophenolate mofetil. Life-long maintenance treatment is required for the majority of patients, but the few who achieve prolonged and stringent biochemical remission with lower alanine aminotransferase and IgG within the normal range may be able to discontinue the medications. In the future, the quality of life of AIH patients should be managed by personalized medicine, including the appropriate selection and dosing of first-line therapy and perhaps alternating with potential therapeutics, and the prediction of the success of treatment withdrawal.

Keywords: Autoimmune hepatitis; Diagnosis; Treatment.

Figure 1.

A schematic model of the progression and transition of AIH. Acute-onset and acute exacerbation of AIH may resolve to chronic AIH, if not all. DILI, drug-induced liver injury; DIAIH, drug-induced autoimmune hepatitis; NAFLD, non-alcoholic fatty liver disease; AIH, autoimmune hepatitis; AS, acute severe; ALF, acute liver failure.

Figure 2.

The treatment response-guided management of AS-AIH. AS, acute severe; AIH, autoimmune hepatitis; MELD, model for end-stage liver disease; HE, hepatic encephalopathy; LT, liver transplantation; ALF, acute liver failure.

Figure 3.

The treatment response-guided management of AIH, excluding AS-AIH and ALF-AIH. AIH, autoimmune hepatitis; AS, acute severe; ALF, acute liver failure.