Immunoglobulin G4-related gastrointestinal diseases, are they immunoglobulin G4-related diseases?

Satomi Koizumi, Terumi Kamisawa, Sawako Kuruma, Taku Tabata, Kazuro Chiba, Susumu Iwasaki, Yuka Endo, Go Kuwata, Koichi Koizumi, Tooru Shimosegawa, Kazuichi Okazaki, Tsutomu Chiba

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41 Citations (Scopus)


In immunoglobulin G4 (IgG4)-related disease (RD), organ enlargement or nodular lesions consisting of abundant infiltration of lymphocytes and IgG4-positive plasma cells and fibrosis are seen in various organs. Although infiltration of many IgG4-positive plasma cells is detected in the gastric and colonic mucosa and major duodenal papilla of patients with autoimmune pancreatitis, it cannot be diagnosed as a gastrointestinal lesion involved in IgG4-RD, because none of the following is observed in these lesions: a mass-like formation; dense fibrosis; or obliterative phlebitis. Based on our review of the literature, there appear to be two types of IgG4-related gastrointestinal disease. One is a gastrointestinal lesion showing marked thickening of the wall of the esophagus and stomach, consisting of dense fibrosis with abundant infiltration of IgG4-positive plasma cells, which usually show submucosal spreading. The other is an IgG4-related pseudotumor occurring in gastrointestinal regions such as the stomach, colon, and major duodenal papilla, showing polypoid or mass-like lesions. Most solitary IgG4-related gastrointestinal lesions that are not associated with other IgG4-RD appear to be difficult to diagnose. It is of utmost importance to rule out malignancy. However, these lesions may respond to steroid therapy. To avoid unnecessary resection, IgG4- related gastrointestinal diseases should be considered in the differential diagnosis.

Original languageEnglish
Pages (from-to)5769-5774
Number of pages6
JournalWorld Journal of Gastroenterology
Issue number35
Publication statusPublished - 2013 Sep 21


  • Autoimmune pancreatitis
  • Colonic polyp
  • Gastritis
  • Immunoglobulin G4
  • Ulcerative colitis

ASJC Scopus subject areas

  • Gastroenterology


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