Editorial
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World J Gastroenterol. Sep 21, 2013; 19(35): 5769-5774
Published online Sep 21, 2013. doi: 10.3748/wjg.v19.i35.5769
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
Satomi Koizumi, Terumi Kamisawa, Sawako Kuruma, Taku Tabata, Kazuro Chiba, Susumu Iwasaki, Yuka Endo, Go Kuwata, Koichi Koizumi, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo 113-8677, Japan
Tooru Shimosegawa, Division of Gastroenterology, Tohoku University, Sendai 980-8574, Japan
Kazuichi Okazaki, Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata 573-1191, Japan
Tsutomu Chiba, Department of Gastroenterology, Kyoto University, Kyoto 606-8507, Japan
Author contributions: Koizumi S and Kamisawa T contributed equally to this work, analyzed the data and wrote the manuscript; Kuruma S, Tabata T, Chiba K, Iwasaki S, Endo Y, Kuwata G and Koizumi K searched the literature; Shimosegawa T, Okazaki K and Chiba T evaluated and edited the manuscript.
Supported by Health and Labour Sciences Research Grants for Research on Intractable diseases (Research on IgG4-related disease) from Ministry of Health, Labour and Welfare of Japan
Correspondence to: Terumi Kamisawa, MD, PhD, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. kamisawa@cick.jp
Telephone: +81-3-38232101 Fax: +81-3-38241552
Received: April 11, 2013
Revised: June 15, 2013
Accepted: July 18, 2013
Published online: September 21, 2013
Processing time: 163 Days and 0.5 Hours
Abstract

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.

Keywords: Immunoglobulin G4; Autoimmune pancreatitis; Gastritis; Colonic polyp; Ulcerative colitis

Core tip: Although the concept of immunoglobulin G4 (IgG4)-related gastrointestinal disease remains unclear, 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. It is of utmost importance to rule out malignancy. To avoid unnecessary resection, IgG4-related gastrointestinal diseases should be considered in the differential diagnosis.