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.

Etiopathogenesis and Diagnostic Strategies in Autoimmune Hepatitis

“…The disease is considerably less frequent in Japan. A trend towards increased prevalence has been observed all over the world [11][12][13]. AIH was originally described in adolescent women.…”

“…Histopathological remission results from biochemical response. Of note, sustaining biochemical remission for a long time (>1 year from the application of treatment) constitutes a surrogate for satisfying overall long-term survival [13]. The first-line paradigm is based on corticosteroids in monotherapy or in combination with azathioprine (AZA).…”

“…Budesonide is contraindicated in cirrhotic patients because of portosystemic shunting which may reduce the drug's efficacy. The combination of AZA with either predniso(lo)ne or budesonide is now believed to be the most standard first-line therapy of AIH in western countries [13,125]. As corticosteroids are still the mainstay of the first-line treatment of AIH, limiting corticosteroids-related osteoporosis in patients with risk factors is required during treatment to promote bone preservation [126].…”

Pathogenesis of autoimmune hepatitis

“…The incidence of AIH induced by methyldopa is 55%, and 43% for hydralazine. A prospective study of the Drug-induced Liver Injury Network (DILIN) showed that nitrofurantoin and nonsteroidal anti-inflammatory drugs accounted for 84% of DIAIH cases, and nitrofurantoin cases were as high as 67%[ 28 , 29 ]. Biological agents ( e.g.…”

The major diagnostic role of autoantibodies in the diagnosis of autoimmune hepatitis, a disease of all ages

“…I read with great interest the very comprehensive review article by Komori who summarized the recent updates regarding the management of autoimmune hepatitis (AIH), focusing on the manifestations of the disease, its diagnosis and treatment [ 1 ].…”