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Autoimmune Liver Diseases: Overlap Syndromes

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Liver Disorders

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Abstract

Overlap syndromes have features of different immune-mediated liver diseases. Findings of autoimmune hepatitis may occur in patients with primary biliary cholangitis or primary sclerosing cholangitis, and patients with primary biliary cholangitis may have features of primary sclerosing cholangitis. Diagnostic criteria have not been codified, but guidelines for diagnosing the overlap syndrome between autoimmune hepatitis and primary biliary cholangitis have been promulgated. Overlap syndromes may respond variably to the conventional treatments for classical disease. Combination therapy with corticosteroids and low-dose ursodeoxycholic acid has been recommended for most patients. Treatment can be individualized and directed to the predominant disease component, and patients may respond to corticosteroids or ursodeoxycholic acid alone. Outcomes are variable and possibly reflect the severity of the cholestatic component. Median graft (10 years) and survival (11 years) after liver transplantation is comparable to results in other immune-mediated liver diseases, but recurrence is more common (53–69 % after 5–10 years).

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Acknowledgement

This review did not receive financial support from a funding agency or institution, and Albert J. Czaja, MD has no conflict of interests to declare.

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  1. Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 First Street S.W., Rochester, MN, 55905, USA

    Albert J. Czaja M.D.

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  1. Albert J. Czaja M.D.
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Correspondence to Albert J. Czaja M.D. .

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  1. Division of Gastroenterology and Hepatology, Medical College of Wisconsin , Milwaukee, Wisconsin, USA

    Kia Saeian

  2. Division of Gastroenterology and Hepatology, Medicine Medical College of Wisconsin , Milwaukee, Wisconsin, USA

    Reza Shaker

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Czaja, A.J. (2017). Autoimmune Liver Diseases: Overlap Syndromes. In: Saeian, K., Shaker, R. (eds) Liver Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-30103-7_18

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  • DOI: https://doi.org/10.1007/978-3-319-30103-7_18

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