IgG4-Related Hepatopathy

  • Takeji Umemura
  • Yoh Zen


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.”


Autoimmune Hepatitis Sclerosing Cholangitis Hepatic Lesion Hepatic Parenchyma Portal Area 
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Copyright information

© Springer Japan 2014

Authors and Affiliations

  1. 1.Division of Gastroenterology and Hepatology, Department of MedicineShinshu University School of MedicineMatsumotoJapan
  2. 2.Institute of Liver StudiesKing’s College HospitalLondonUK

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