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Managing the Patient with Features of Overlapping Autoimmune Liver Disease

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Autoimmune Hepatitis

Part of the book series: Clinical Gastroenterology ((CG))

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Abstract

Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC) (including small duct PSC) as a group are commonly referred to as “autoimmune liver diseases.” They all have characteristic features, and the differential diagnosis between them is usually straightforward. Corticosteroids alone or in combination with azathioprine improve survival in AIH. Ursodeoxycholic acid (UDCA) delays disease progression in PBC. No medical therapy is currently recommended for PSC although UDCA often improves liver biochemistry and hence is used to some extent. Some patients present with clinical, biochemical, serological, and/or histological characteristics of both a cholestatic liver disease and AIH. Sequential development of characteristics of two diseases has also been described. These variant conditions are often designated PBC–AIH- and PSC–AIH “overlap syndromes,” respectively. Approximately 10% of patient cohorts with a primary diagnosis of PBC or PSC have been classified as “overlap syndromes” in various reports. However, standardized criteria for “overlap syndromes” are lacking, and they are not defined diagnostic entities. Randomized, controlled therapeutic trials are impossible to conduct due to patient heterogeneity and low numbers. Treatment recommendations therefore are not evidence-based. Efforts should always be made to define the primary disorder and therapy given accordingly. Some patients with overlapping features of PBC and AIH appear to benefit from a combination of UDCA and corticosteroids. Treatment should be individualized, with awareness of the risk of side effects. Similarly, corticosteroid (±azathioprine) therapy should be considered in selected PSC patients with clear features of AIH.

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  1. Oslo University Hospital, Rikshospitalet, 4950, Nydalen, 0424, Oslo, Norway

    Kirsten Muri Boberg

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  1. Toronto Western Hospital Liver Centre, Department of Medicine, University of Toronto, Bathhurst St. 399, Toronto, M5T 2S8, Ontario, Canada

    Gideon M. Hirschfield

  2. Toronto Western Hospital Liver Centre, University of Toronto, Bathhurst St. 399, Toronto, M5T 2S8, Ontario, Canada

    E. Jenny Heathcote

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Boberg, K.M. (2012). Managing the Patient with Features of Overlapping Autoimmune Liver Disease. In: Hirschfield, G., Heathcote, E. (eds) Autoimmune Hepatitis. Clinical Gastroenterology. Springer, New York, NY. https://doi.org/10.1007/978-1-60761-569-9_12

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