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IgG4-Related Autoimmune Hepatitis

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Autoimmune Liver Diseases

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Abstract

Autoimmune hepatitis (AIH) has been broadly categorized into two distinct disease subtypes on the basis of antibody profiles. In some patients with definite AIH, we have observed abnormal serum concentrations of immunoglobulin G4 (IgG4) and extensive IgG4-positive plasma cell infiltration in liver biopsy samples. We propose that such cases should fall under a new clinicopathological entity termed “IgG4-related AIH.” In this chapter, we first introduce our study on abnormal liver biopsy specimens in autoimmune pancreatitis before defining the concept of IgG4-related hepatopathy. Next, we present several cases that meet the criteria of IgG4-related AIH. Since IgG4-related AIH has a low incidence of approximately 3 %, it is still controversial whether this entity truly exists. However, we lastly describe a new possible association between posttransplant de novo AIH/plasma cell hepatitis and IgG4-related disease.

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Authors and Affiliations

  1. Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, 390-8621, Japan

    Takeji Umemura

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  1. Takeji Umemura
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Correspondence to Takeji Umemura .

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Editors and Affiliations

  1. Dept of Gastroenterology & Rheumatology, Fukushima Medical University of Medicine, Fukushima, Japan

    Hiromasa Ohira

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Umemura, T. (2014). IgG4-Related Autoimmune Hepatitis. In: Ohira, H. (eds) Autoimmune Liver Diseases. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54789-1_11

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  • DOI: https://doi.org/10.1007/978-4-431-54789-1_11

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54788-4

  • Online ISBN: 978-4-431-54789-1

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