The Asian Pacific Association for the Study of the Liver clinical practice guidance: the diagnosis and management of patients with autoimmune hepatitis

First Results From a Propensity Matching Trial of Mycophenolate Mofetil vs. Azathioprine in Treatment-Naive AIH Patients

“…Autoimmune hepatitis (AIH) is a disease of unknown etiology characterized by distinct elevation of serum immunoglobulin G (IgG) levels even in patients without cirrhosis, female predominance, non-organ-and/or organ-specific autoantibodies, interface hepatitis on histology, and favorable response to immunosuppressive treatment (1)(2)(3)(4)(5)(6). Administration of corticosteroids with or without azathioprine (AZA) is considered the standard of care as first-line therapy (2)(3)(4)(5)(6).…”

“…Autoimmune hepatitis (AIH) is a disease of unknown etiology characterized by distinct elevation of serum immunoglobulin G (IgG) levels even in patients without cirrhosis, female predominance, non-organ-and/or organ-specific autoantibodies, interface hepatitis on histology, and favorable response to immunosuppressive treatment (1)(2)(3)(4)(5)(6). Administration of corticosteroids with or without azathioprine (AZA) is considered the standard of care as first-line therapy (2)(3)(4)(5)(6). The aims of such treatment are to achieve complete biochemical response (CBR; normal transaminases and IgG) and histological remission of the disease [modified hepatitis activity index (mHAI <4)] and, eventually, prevent fibrosis progression and development of end-stage liver disease at the minimal risk of side effects of therapy (2)(3)(4)(5)(6).…”

“…Administration of corticosteroids with or without azathioprine (AZA) is considered the standard of care as first-line therapy (2)(3)(4)(5)(6). The aims of such treatment are to achieve complete biochemical response (CBR; normal transaminases and IgG) and histological remission of the disease [modified hepatitis activity index (mHAI <4)] and, eventually, prevent fibrosis progression and development of end-stage liver disease at the minimal risk of side effects of therapy (2)(3)(4)(5)(6).…”

“…Nevertheless, in a recent retrospective analysis, it has been shown that determination of AZA metabolite levels could improve biochemical response rates with fewer adverse events (17). Concisely, the standard treatment bares about 15%-25% rates of no response or intolerance to AZA, while patients who are not able to achieve predniso(lo)ne withdrawal and remained at doses higher than 10 mg/day for more than 18 months may suffer several corticosteroid-related complications (2)(3)(4)(5)(6). All together, these data could suggest that predniso(lo)ne with or without AZA is far from the ideal treatment option for AIH (15).…”

Impact of genetic and environmental factors on autoimmune hepatitis

“…Due to the absence of a single diagnostic marker, AIH diagnosis is based on diagnostic criteria comprising of a combination of four parameters namely, IgG, the presence of autoantibodies, liver histology and absence of viral hepatitis markers [ 9 ]. The disease is further sub-typed in AIH-type 1, characterized by the presence of antinuclear antibodies (ANA) and anti-smooth muscle antibodies (SMA) and AIH-type 2, which predominantly affects children and adolescents and is characterized by the presence of anti-liver kidney microsomal type 1 (anti-LKM1) and/or anti-LKM type 3 (anti-LKM3)antibodies, with or without anti-liver cytosol type 1 (anti-LC1) antibodies [ [10] , [11] , [12] , [13] , [14] ].…”