Kenji Notohara1, Terumi Kamisawa2, Kazushige Uchida3, Yoh Zen4, Mitsuhiro Kawano5, Satomi Kasashima6, Yasuharu Sato7, Masahiro Shiokawa8, Takeshi Uehara9, Hajime Yoshifuji10, Hiroko Hayashi11, Koichi Inoue12, Keisuke Iwasaki13, Hiroo Kawano14, Hiroyuki Matsubayashi15, Yukitoshi Moritani16, Katsuhiko Murakawa17, Yoshio Oka18, Masatoshi Tateno19, Kazuichi Okazaki3, Tsutomu Chiba8. 1. Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan. 2. Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan. kamisawa@cick.jp. 3. Department of Gastroenterology and Hepatology, Kansai Medical University, Hirakata, Osaka, Japan. 4. Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan. 5. Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan. 6. Department of Pathology, National Hospital Organization, Kanazawa Medical Center, Kanazawa, Japan. 7. Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan. 8. Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 9. Department of Laboratory Medicine, Shinshu University School of Medicine, Matsumoto, Japan. 10. Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. 11. Department of Pathology, Nagasaki University Graduate School of Biomedical Science, Nagasaki, Japan. 12. Division of Surgery, Yamachika Memorial Hospital, Odawara, Kanagawa, Japan. 13. Department of Pathology, Sasebo City General Hospital, Sasebo, Japan. 14. Department of Laboratory Science, Faculty of Health Science, Yamaguchi University Graduate School of Medicine, Ube, Japan. 15. Division of Endoscopy, Shizuoka Cancer Center, Suntogun, Shizuoka, Japan. 16. Moritani Surgical Clinic, Okayama, Japan. 17. Department of Surgery, Obihiro Kosei General Hospital, Obihiro, Japan. 18. Department of Surgery, Nishinomiya Municipal Central Hospital, Nishinomiya, Japan. 19. Department of Pathology, Kushiro Red Cross Hospital, Kushiro, Japan.
Abstract
BACKGROUND: Several reports on immunoglobulin (Ig)G4-related disease (IgG4-RD) with gastrointestinal involvement (IgG4-related gastrointestinal disease; IgG4-GID) have been published, although this entity has not been fully established clinicopathologically. Thus, we carried out a multicenter survey. METHODS: Patients with possible IgG4-GID who underwent resection were collected. Histologic slides were reevaluated, and eight cases with diffuse lymphoplasmacytic infiltration but without numerous neutrophils, granulations or epithelioid granulomas were further analyzed. RESULTS: Overall, the IgG4 counts (87-345/high-power field) and IgG4/IgG-positive ratio were high (44-115%). The demographic findings included advanced age among the patients (55-80 years) and male preponderance (six cases). Six lesions (five gastric, one esophageal), consisting of lymphoplasmacytic infiltration with neural involvement in the muscularis propria and/or bottom-heavy plasmacytosis in the gastric mucosa, were histologically regarded as highly suggestive of IgG4-RD. Storiform fibrosis and obliterative phlebitis were found in two cases, and the former gave rise to a 7-cm-sized inflammatory pseudotumor (IPT) in one case. Ulceration and carcinoma co-existed in three and two lesions, respectively. All the patients had other organ involvement (OOI), and serum IgG4 levels were markedly elevated (four of five patients). The remaining two cases with gastric IPTs featuring reactive nodular fibrous pseudotumor or nodular lymphoid hyperplasia were regarded as possible cases of IgG4-RD because of the histologic findings and lack of OOI. CONCLUSIONS: IgG4-GID is found in the setting of IgG4-RD, often with ulceration or cancer. Characteristic histologic findings are observed in the muscularis propria and gastric mucosa. Cases with IPT may be heterogeneous, and there may be mimickers of IgG4-GID.
BACKGROUND: Several reports on immunoglobulin (Ig)G4-related disease (IgG4-RD) with gastrointestinal involvement (IgG4-related gastrointestinal disease; IgG4-GID) have been published, although this entity has not been fully established clinicopathologically. Thus, we carried out a multicenter survey. METHODS:Patients with possible IgG4-GID who underwent resection were collected. Histologic slides were reevaluated, and eight cases with diffuse lymphoplasmacytic infiltration but without numerous neutrophils, granulations or epithelioid granulomas were further analyzed. RESULTS: Overall, the IgG4 counts (87-345/high-power field) and IgG4/IgG-positive ratio were high (44-115%). The demographic findings included advanced age among the patients (55-80 years) and male preponderance (six cases). Six lesions (five gastric, one esophageal), consisting of lymphoplasmacytic infiltration with neural involvement in the muscularis propria and/or bottom-heavy plasmacytosis in the gastric mucosa, were histologically regarded as highly suggestive of IgG4-RD. Storiform fibrosis and obliterative phlebitis were found in two cases, and the former gave rise to a 7-cm-sized inflammatory pseudotumor (IPT) in one case. Ulceration and carcinoma co-existed in three and two lesions, respectively. All the patients had other organ involvement (OOI), and serum IgG4 levels were markedly elevated (four of five patients). The remaining two cases with gastric IPTs featuring reactive nodular fibrous pseudotumor or nodular lymphoid hyperplasia were regarded as possible cases of IgG4-RD because of the histologic findings and lack of OOI. CONCLUSIONS: IgG4-GID is found in the setting of IgG4-RD, often with ulceration or cancer. Characteristic histologic findings are observed in the muscularis propria and gastric mucosa. Cases with IPT may be heterogeneous, and there may be mimickers of IgG4-GID.
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