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IgG4 associated autoimmune hepatitis: a differential diagnosis for classical autoimmune hepatitis
  1. T Umemura1,
  2. Y Zen2,
  3. H Hamano3,
  4. T Ichijo3,
  5. S Kawa4,
  6. Y Nakanuma5,
  7. K Kiyosawa6
  1. 1Department of Internal Medicine, Division of Hepatology and Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan
  2. 2Division of Pathology, Kanazawa University Hospital, Kanazawa, Japan
  3. 3Department of Internal Medicine, Division of Hepatology and Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan
  4. 4Centre for Health, Safety and Environmental Management, Shinshu University, Matsumoto, Japan
  5. 5Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
  6. 6Department of Internal Medicine, Division of Hepatology and Gastroenterology, Shinshu University School of Medicine, Matsumoto, Japan
  1. Correspondence to:
    Dr Takeji Umemura
    Department of Internal Medicine, Division of Hepatology and Gastroenterology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390–8621, Japan; t-ume{at}hsp.md.shinshu-u.ac.jp

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Autoimmune pancreatitis is characterised by raised serum levels of IgG4 and IgG4-bearing plasma cells in the inflammatory tissue.1 Similar pathology can also occur in the larger bile ducts, resembling sclerosing cholangitis.2 We report a patient with hepatitis and chronic cholecystitis, raised IgG4 level, and IgG4-bearing plasma cells in the liver and gall bladder wall, but no evident pancreatic disease.

In January 2003, a cholecystectomy was undertaken on a 54 year old woman for chronic cholecystitis. The extracted gall bladder showed lymphoplasmacytic infiltration and subserosal fibrosis. A liver biopsy was done because of abnormal liver function tests and showed severe lobular hepatitis with mild portal inflammation. Plasma cell infiltration was observed in the portal tracts and parenchyma, though fibrosis was not significant. IgG4 bearing plasma cell infiltration was found both in the liver and the gall bladder wall. After six months, liver function was still abnormal: aspartate …

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