Diagnosis and Therapy of Autoimmune Hepatitis

Abstract

Autoimmune hepatitis (AIH) is a chronic progressive hepatitis, characterized by interface hepatitis with lymphoplasmacellular infiltrates on liver biopsy, high serum globulin level and circulating autoantibodies. It is classified into two types, according to autoantibody profile: type 1 is characterized by anti-nuclear (ANA) and/or anti-smooth muscle (SMA) antibodies; type 2 by anti-liver kidney microsomal type 1 (anti-LKM-1) antibodies. AIH affects all ages, may be asymptomatic, frequently has an acute onset, and can present as fulminant hepatitis. The diagnosis of AIH is based on a scoring system codified by an international consensus. Corticosteroids alone or in conjunction with azathioprine is the treatment of choice in patients with AIH and results in remission induction in over 80% of patients. Alternative proposed strategies in patients who have failed to achieve remission on standard therapy or patients with drug toxicity include the use of cyclosporine, tacrolimus, budesonide or mycophenolate mofetil. Liver transplantation is the treatment of choice in managing decompensated disease, however AIH can recur or develop de novo after liver transplantation.

Celiac Disease and Elevated Liver Enzymes: A Still Not Fully Defined Pathogenesis

“…8 These autoantibodies, although of IgG class, are also known to have very high specificity for AIH. 7,9 This similarity between the two autoimmune diseases could be a clue that also supports possible immune-mediated pathogenesis of hypertransaminasemia in CD patients. Therefore, it would be relevant and worthy of study to analyze the presence of anti-actin antibodies in CD patients to verify whether these antibodies are markers of liver injury.…”

“…[3][4][5][6] Among the autoimmune diseases potentially associated with CD, autoimmune hepatitis (AIH) is worthy of mention, as previously reported. 1,3,7 Of considerable interest, it has been reported that celiac patients frequently have anti-filamentous actin IgA antibodies that have shown reliable and significant correlation with villous atrophy. 8 These autoantibodies, although of IgG class, are also known to have very high specificity for AIH.…”

“…Among the autoimmune diseases potentially associated with CD, autoimmune hepatitis (AIH) is worthy of mention, as previously reported. 1 , 3 , 7 …”

“… 8 These autoantibodies, although of IgG class, are also known to have very high specificity for AIH. 7 , 9 …”

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

“…Lastly, a further entity characterized by autoantibody negativity, but exhibiting the same clinical/laboratory features of the classic AIH and the typical response to immunosuppressive therapy, has been hypothesized. [2][3][4] The search for autoantibodies is a crucial step in the diagnosis of AIH and needs to be stressed because there are still many differences in the world regarding the familiarity of physicians and laboratories with these autoantibodies. 5 The proper knowledge of autoantibodies and the optimal methods for their research allows to correctly diagnose chronic liver diseases initially classified as cryptogenic.…”