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Autoimmune Hepatitis

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Clinical Cases in Hepatology

Abstract

An abnormal liver profile characterized by increase serum activity of alanine and aspartate aminotransferases, variable degrees of hyperbilirubinemia, high serum concentrations of IgG, and autoantibodies characterize autoimmune hepatitis (AIH). The disease is more common in women than in men. The histological features of AIH include interface hepatitis with lymphoplamacytic infiltrate, lobular necrosis, and rosette formation.

The etiology of AIH is unknown. Immunological alterations considered relevant in the disease’s etiology include altered number and function of regulatory T cells, gamma delta T cells as effectors, chemokines, intestinal dysbiosis, environmental factors, and molecular mimicry. Epigenetics with the role of microRNAs in the pathogenesis of the disease has been recently proposed.

Genetic susceptibility related to the human leukocyte antigen class I and genetic polymorphisms outside the major histocompatibility complex has been identified.

The treatment of autoimmune hepatitis is corticosteroids alone or in combination with azathioprine. The goal is to suppress the inflammatory process, reflected by normalization of serum liver profile and markers of autoimmune activity, i.e., serum gammaglobulin concentrations. Maintenance therapy with azathioprine alone beyond the first few weeks of treatment has been proposed to spare steroids' side effects. There is experience with budesonide to limit the systemic exposure to the steroid effects. Patients who do not respond or do not tolerate standard therapy can be managed with calcineurin inhibitors, cyclosporine, and tacrolimus, and the reversible inhibitor of inosine monophosphate dehydrogenase in purine synthesis, mycophenolate mofetil.

Patients with AIH may require liver transplantation. AIH recurs in the liver graft.

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Acknowledgments

The author acknowledges Dr. Cesar del Rosario for having contributed Figures 4.1, 4.2, and 4.3.

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Bergasa, N.V. (2022). Autoimmune Hepatitis. In: Bergasa, N.V. (eds) Clinical Cases in Hepatology. Springer, London. https://doi.org/10.1007/978-1-4471-4715-2_4

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