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IgG4-Related Hepatopathy

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IgG4-Related Disease

Abstract

In this chapter, we introduce the hepatic lesions of IgG4-related disease. We previously conducted a detailed study on hepatic parenchyma biopsy specimens in autoimmune pancreatitis (AIP) and proposed the concept of “IgG4 hepatopathy,” now termed IgG4-related hepatopathy. Inflammatory lesions of IgG4-related hepatopathy involve not only the biliary system but also extend to the hepatic parenchyma. In addition, we observe that in some patients who meet the diagnostic criteria for autoimmune hepatitis (AIH), abnormal serum concentrations of IgG4 are detected and copious numbers of IgG4-positive plasma cells are found within liver biopsy samples. We propose that some of these cases have a clinicopathological entity that is most appropriately termed “IgG4-related AIH.”

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Correspondence to Takeji Umemura .

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© 2014 Springer Japan

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Umemura, T., Zen, Y. (2014). IgG4-Related Hepatopathy. In: Umehara, H., Okazaki, K., Stone, J., Kawa, S., Kawano, M. (eds) IgG4-Related Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54228-5_29

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  • DOI: https://doi.org/10.1007/978-4-431-54228-5_29

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54227-8

  • Online ISBN: 978-4-431-54228-5

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