Recent advances in the diagnosis and management of autoimmune hepatitis

Abstract

Autoimmune hepatitis (AIH) is an acute or chronic inflammatory disease of the liver caused by an unclear immune response that affects people from all ethnic groups irrespective of age or sex. It is characterized by hyperglobulinemia, circulating autoantibodies and liver inflammation. The clinical picture of the disease varies from asymptomatic and/or mild to severe acute hepatitis or liver failure. A timely and prompt diagnosis is of utmost importance to prevent progression to advanced liver disease by immediate initiation of immunosuppressive treatment. So far, several diagnosing scoring systems have incorporated the demographics, biochemical, clinical, and histological characteristics of the disease.However, due to the high heterogeneity of the disease presentation, AIH diagnosis remains challenging. Most patients initially respond to first-line treatment, which consists of corticosteroids combined with azathioprine while some authorities also suggest the use of mycophenolate mofetil instead. However, insufficient response and intolerance due to side effects are common, so a significant proportion of patients requires second-and/or third-line therapies. Herein, we review the challenges and recent advances in AIH diagnosis and management.

The Role of the NLRP3 Inflammasome and Programmed Cell Death in Acute Liver Injury

“…Autoimmune hepatitis (AIH), a complex hepatic inflammatory disease mediated by autoimmune reactions, can present in both acute and chronic forms [ 165 ]. Its clinical features are characterized by elevated serum transaminases, high γ- Hypogammaglobulinemia, autoantibody positivity, and interface hepatitis, with a predominance of lymphocytic and plasma cell infiltrates [ 166 ].…”

Denosumab‐induced autoimmune hepatitis: Case report

“…The immunopathogenesis of AIH is based on autoreactive CD4 and CD8 T cells, which are linked to the body's impaired immunoregulatory mechanisms that result in the loss of self‐tolerance to antigens on hepatocytes, which causes autoreactive T cells to destroy hepatic parenchyma 3,4 . The clinical presentation of AIH can range from entirely asymptomatic to experiencing general symptoms (such as malaise, fatigue, anorexia, arthralgias and weight loss) 5,6 …”

“…3,4 The clinical presentation of AIH can range from entirely asymptomatic to experiencing general symptoms (such as malaise, fatigue, anorexia, arthralgias and weight loss). 5,6 To diagnose AIH, a liver biopsy and compatible histology findings are necessary. In addition, it is crucial to evaluate specific clinical and laboratory findings, including elevated levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT), as well as serum IgG, and the presence of specific autoantibodies such as antinuclear antibodies, SMA, and anti-LKM1 3,7 Hepatotoxicity induced by drugs can either be a predictable, dose-related reaction, or it usually results from direct toxicity of the drug or its metabolite.…”

“… 3 , 4 The clinical presentation of AIH can range from entirely asymptomatic to experiencing general symptoms (such as malaise, fatigue, anorexia, arthralgias and weight loss). 5 , 6 …”

Inflammasome and pyroptosis in autoimmune liver diseases

“…Autoimmune liver diseases (AILDs) are a group of chronic liver diseases caused by immune dysfunction ( 1 ), including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), IgG4-related sclerosing cholangitis (IgG4-SC), and the so-called overlap syndrome ( 2 ). In AIH, hepatocytes undergo pathological alterations, with high serum levels of transaminase and IgG (or gamma globulin) and positive serum antinuclear and smooth muscle antibodies ( 3 ).…”