Immunoglobulin G4-related Gastrointestinal Disease Terumi Kamisawa 1 , Sawako Kuruma 1 , Kazuro Chiba 1 , Satomi Koizumi 1 , Kensuke Yoshimoto 1 , Masataka Kikuyama 1 , Kenji Notohara 2 1Department of Gastroenterology, Tokyo Metropolitan Komagome Hospital, Tokyo 2Department of anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan Keyword: IgG4 , IgG4関連疾患 , 形質細胞 , 消化管 pp.1709-1714
Published Date 2019/12/25
DOI https://doi.org/10.11477/mf.1403201902
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 The concept of IgG4-GID(immunoglobulin G4-related gastrointestinal disease)is yet not established. Reported cases of IgG4-GID were divided into gastrointestinal lesions showing marked thickening of the stomach wall and esophagus consisting of dense fibrosis with abundant infiltration of IgG4-positive plasma cells, which usually present as a submucosal layer, and IgG4-related pseudotumor occurring in the stomach and colon. Nodular formation or wall thickening with abundant infiltration of IgG4-positive plasma cells is essential for the diagnosis of IgG4-GID. However, for the diagnosis, the characteristic histological features, such as storiform fibrosis and obliterative phlebitis, elevated serum IgG4 levels, and other IgG4-related diseases should be fully considered. Strands of dense lymphoplasmacytic infiltration within the thickened muscularis propria and aggregation of plasma cells in the deeper mucosa might also be the characteristic histological features of IgG4-GID.

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