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Autoimmune Hepatitis and Overlap Syndromes

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Liver Disease in Clinical Practice

Part of the book series: In Clinical Practice ((ICP))

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Autoimmune hepatitis (AIH) is an infrequent, characteristically corticosteroid-responsive, chronic lifelong liver disease, impacting all ages and both sexes. In the majority of cases, treatment with immunosuppressants is effective and has been shown to prolong survival, particularly when patients are diagnosed promptly and managed carefully. Although practical diagnostic criteria exist, diagnosis of AIH often remains a challenge given the heterogeneity of disease presentation, the broad differential diagnosis, and absence of definitive specific investigations. In many patients, treatment with conventional immunosuppressants remains inadequate to control disease fully and/or related treatment side effects impede adherence, collectively leading to poorer outcomes than ideal. Approaches to overcome this are diverse without complete clinical alignment, e.g., particularly in the use of third-line therapies. A more standardized approach to management and therapies with fewer side effects remains needed for all patients. Further understanding of the aetiology of AIH—particularly relating to immunoregulatory pathways spanning T and B cell function—may lead to development in time of targeted therapies, which may prove especially valuable if they help re-establish immune homeostasis without the need for lifelong treatment.

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Correspondence to Gideon M. Hirschfield .

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Leung, K.K., Hirschfield, G.M. (2022). Autoimmune Hepatitis and Overlap Syndromes. In: Cross, T. (eds) Liver Disease in Clinical Practice. In Clinical Practice. Springer, Cham.

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  • Print ISBN: 978-3-031-10011-6

  • Online ISBN: 978-3-031-10012-3

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