Gastric involvement in IgG4-related disease

Compromiso gástrico por enfermedad relacionada con IgG4

Introduction: IgG4-related disease (IgG4-RD) is a recently described entity, characterized by IgG4+ infiltration of plasma cells and storiform pattern sclerosis mediated by CD4+ lymphocytes and follicular helper T-cells (Tfhs). Most patients present with adenopathy and weight loss. Among the most affected sites are the pancreas, salivary and lacrimal glands, aorta, and retroperitoneum. Gastric involvement is rare. Diagnosis is based on histopathological examination of the involved organ, clinical manifestations, and serological results. Glucocorticoid therapy is the first line management. Case report: a 38-year-old male patient with symptoms suggestive of upper gastrointestinal (GI) bleeding, upper GI endoscopy revealed a gastric corpus ulcer, suspicious of an ulcerated gastric subepithelial lesion, possibly a gastrointestinal stromal tumor (GIST). Consequently, laparoscopic subtotal gastrectomy was performed. Histology was compatible with an ulcerated IgG4-related fibrosclerosing tumor. Glucocorticoid therapy was started with patient´s favorable response. Conclusion: in this paper, we will review the cases of IgG4-RD with gastric involvement reported in the literature to describe the most important features of this condition.

1. Maritati F, Peyronel F, Vaglio A. IgG4-related disease: a clinical perspective. Rheumatology (Oxford). 20201;59(Suppl3):iii123-iii131. https://doi.org/10.1093/rheumatology/kez667
2. Perugino CA, AlSalem SB, Mattoo H, Della -Torre E, Mahajan V, Ganesh G, Allard-Chamard, et al. Identification of galectin-3 as an autoantigen in patients with IgG4-related disease. J Allergy Clin Immunol. 2019;143(2):736-745.e6. https://doi.org/10.1016/j.jaci.2018.05.011.
3. Shiokawa M, Kodama Y, Sekiguchi K, Kuwada T, Tomono T, Kuriyama K et al. Laminin 511 is a target antigen in autoimmune pancreatitis. Sci Transl Med. 2018;10(453):eaaq0997. https://doi.org/10.1126/scitranslmed.aaq0997.
4. Hubers LM, Vos H, Schuurman AR, Erken R, Oude Elferink RP, Burgering B, et al. Annexin A11 is targeted by IgG4 and IgG1 autoantibodies in IgG4-related disease. Gut. 2018;67(4):728-735. https://doi.org/10.1136/gutjnl-2017-314548.
5. Uchida K, Masamune A, Shimosegawa T, Okazaki K. Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009. Int J Rheumatol. 2012;2012:358371. https://doi.org/10.1155/2012/358371.
6. Masamune A, Kikuta K, Hamada S, Tsuji I, Takeyama Y, Shimosegawa T, et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2016. J Gastroenterol. 2020;55(4):462-470. https://doi.org/10.1007/s00535-019-01658-7.
7. Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol. 2020;16(12):702-714. https://doi.org/10.1038/s41584-020-0500-7.
8. Wallace ZS, Naden RP, Chari S, Choi H, Della-Torre E, et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Arthritis Rheumatol. 2020;72(1):7-19. https://doi.org/10.1002/art.41120.
9. Sánchez-Oro R, Alonso-Muñoz EM, Martí Romero L. Revisión de la enfermedad relacionada con la IgG4. Gastroenterol Hepatol. 2019;42(10):638-647. https://doi.org/10.1016/j.gastrohep.2019.08.009.
10. Lanzillotta M, Mancuso G; Della-Torre E. Advances in the diagnosis and management of IgG4 related disease. BMJ. 2020;369:m1067. https://doi.org/10.1136/bmj.m1067.
11. Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23(1):57-66. https://doi.org/10.1097/BOR.0b013e3283418057.
12. Miyabe K, Zen Y, Cornell LD, Rajagopalan G, Chowdhary VR, Roberts LR et al. Gastrointestinal and Extra-Intestinal Manifestations of IgG4-Related Disease. Gastroenterology. 2018;155(4):990-1003. https://doi.org/10.1053/j.gastro.2018.06.082.
13. Umehara H, Okazaki K, Kawa S, Takahashi H, Goto H, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD, Mod Rheumatol. 2021;31(3):529-533. https://doi.org/10.1080/14397595.2020.1859710.
14. Woo CG, Yook JH, Kim AY, Kim J. IgG4-Related Disease Presented as a Mural Mass in the Stomach. J Pathol Transl Med. 2016;50(1):67-70. https://doi.org/10.4132/jptm.2015.07.28.
15. Berger Z, Lea-Plaza MI, Varela C, Becerra M, et al. Gastropatía hipertrófica por IgG4. Reporte del primer caso en Chile. Rev Méd Chile. 2019;147(1):119-124. http://dx.doi.org/10.4067/S0034-98872019000100119.
16. Lim DY, Cheng LT, Yew Tan DM, Jajeh IAI. Isolated IgG4-related gastric disease presenting as diffuse gastric wall thickening with ulcer. J Radiol Case Rep. 2018;12(9):9-20. http://dx.doi.org/10.3941/jrcr.v12i9.3493.
17. Bulanov D, Arabadzhieva E, Bonev S, Yonkov A, et al. A rare case of IgG4-related disease: A gastric mass, associated with regional lymphadenopathy. BMC Surg. 2016;16(1):37. http://dx.doi.org/10.1186/s12893-016-0151-4.
18. Woo CG, Yook JH, Kim AY, Kim J. IgG4-Related Disease Presented as a Mural Mass in the Stomach. J Pathol Transl Med. 2016;50(1):67-70. http://dx.doi.org/10.4132/jptm.2015.07.28.
19. Otsuka R, Kano M, Hayashi H, Hanari N, et al. Probable IgG4-related sclerosing disease presenting as a gastric submucosal tumor with an intense tracer uptake on PET/CT: a case report. Surg Case Rep. 2016;2(1):33. http://dx.doi.org/10.1186/s40792-016-0161-6.
20. Cheong HR, Lee BE, Song GA, Kim GH, An SG, Lim W. Immunoglobulin G4-Related Inflammatory Pseudotumor Presenting as a Solitary Mass in the Stomach. Clin Endosc. 2016;49(2):197-201. http://dx.doi.org/10.5946/ce.2015.074.
21. Skorus U, Kenig J, Mastalerz K. IgG4-related disease manifesting as an isolated gastric lesion- a literature review. Pol Przegl Chir. 2018;90(4):41-45. http://dx.doi.org/10.5604/01.3001.0012.0976.
22. Bohlok A, Khoury ME, Tulelli B, Verset L, Zaarour A, Demetter P, et al. A rare presentation of IgG4 related disease as a gastric antral lesion: Case report and review of the literature. Int J Surg Case Rep. 2018;51:244-247. http://dx.doi.org/10.1016/j.ijscr.2018.08.065.
23. Muto O, Tamakawa S, Takahashi K, Yokohama S, Takasoe A, et al. IgG4-related Disease Manifesting as Gastroduodenal Ulcer Diagnosed by an Endoscopic Biopsy. Intern Med. 2020;59(20):2491-2497. http://dx.doi.org/10.2169/internalmedicine.4483-20.
24. Chen L, Almudaires A, Alzahrani M, Qumosani K, Chakrabarti S. IgG4-related disease as a rare cause of gastric outlet obstruction: a case report and literature review. BMC gastroenterology. 2021;21(1):349. http://dx.doi.org/10.1186/s12876-021-01927-x.
25. Probst A, Schaller T, Sommer F, Geissler B, et al. Immunoglobulin G4 (IgG4)-related disease of the stomach - a challenging differential diagnosis in suspected gastric cancer. Z Gastroenterol. 2019;57(11):1298-1303. http://dx.doi.org/10.1055/a-1013-4437.
26. Ramakrishna B, Yewale R, Vijayakumar K, Radhakrishna P, Ramakrishna BS. Gastric IgG4-related disease presenting as a mass lesion and masquerading as a gastrointestinal stromal tumor. J Pathol Transl Med. 2020;54(3):258-262. http://dx.doi.org/10.4132/jptm.2020.02.10.
27. Zhang X, Jin X, Guan L, Lin X, Li X, Li Y. IgG4-Related Disease With Gastrointestinal Involvement: Case Reports and Literature Review. Front Immunol. 2022;13:816830. http://dx.doi.org/10.3389/fimmu.2022.816830.
28. Seo HS, Jung YJ, Park CH, Song KY, Jung ES. IgG4-related Disease in the Stomach which Was Confused with Gastrointestinal Stromal Tumor (GIST): Two Case Reports and Review of the Literature. J Gastric Cancer. 2018;18(1):99-107. http://dx.doi.org/10.5230/jgc.2018.18.e8.
29. Zhang F, Xu J, Zhu Y, Wu Q, Xie X, Shi Y. Stomach mass” as the first manifestation of IgG4-related disease: a case report. BMC Gastroenterol. 2021;21(1):442. http://dx.doi.org/10.1186/s12876-021-02013-y.
30. Ramakrishna B, Yewale R, Vijayakumar K, Radhakrishna P, Ramakrishna BS. Gastric IgG4-related disease presenting as a mass lesion and masquerading as a gastrointestinal stromal tumor. J Pathol Transl Med. 2020;54(3):258-262. http://dx.doi.org/10.4132/jptm.2020.02.10.
31. Yamane T, Eto K, Morinaga T, Matsumura K, Yamashita K, et al. IgG4-related disease presenting as a submucosal tumor of the stomach resected with laparoscopic endoscopic cooperative surgery: a case report. Surg Case Rep. 2020;6(1):93. http://dx.doi.org/10.1186/s40792-020-00851-8.
32. Ortuño Moreno MI, Ferri Ñíguez B, Martínez Barba E, Fernández Hernández JÁ. An ulcerated gastric ulcer and pseudotumour with pancreatic affectation associated with immunoglobulin G4-related disease: a case report and literature review. Rev Esp Enferm Dig. 2017;109(12):870-874. http://dx.doi.org/10.17235/reed.2017.4996/2017.
33. Inoue K, Okubo T, Kato T, Shimamura K, Sugita T, Kubota K, et al. IgG4-related stomach muscle lesion with a renal pseudotumor and multiple renal rim-like lesions: A rare manifestation of IgG4-related disease. Mod Rheumatol. 2018;28(1):188-192. http://dx.doi.org/10.3109/14397595.2015.1081743.
34. Khan S, Zhu LP, Jiang K, Liu W, Chen X, Wang BM. Immunoglobulin G4-Related Disease Manifesting as Isolated, Typical, and Nontypical Gastroesophageal Lesion: A Research of Literature Review. Digestion. 2020;101(5):506-521. http://dx.doi.org/10.1159/000501513.
35. Lohr JM, Beuers U, Vujasinovic M, Alvaro D, Frokjaer JB, Buttgereit F, et al. European Guideline on IgG4-related digestive disease - UEG and SGF evidence based recommendations. United European Gastroenterol J. 2020;8(6):637-666. http://dx.doi.org/10.1177/2050640620934911.
36. Campochiaro C, Della Torre E, Lanzillotta M, Bozzolo E, Baldissera E, Milani R, et al. Long term efficacy of maintenance therapy with Rituximab for IgG4-related disease. Eur J Intern Med. 2020;74:92-98. http://dx.doi.org/10.1016/j.ejim.2019.12.029.
37. Omar D, Chen Y, Cong Y, Dong L. Glucocorticoids and steroid sparing medications monotherapies or in combination for IgG4-RD: a systematic review and network meta analysis. Rheumatology (Oxford). 2020;59(4):718-726. http://dx.doi.org/10.1093/rheumatology/kez380.
38. Kim HM, Chung MJ, Chung JB. Remission and relapse of autoimmune pancreatitis: focusing on corticosteroid treatment. Pancreas. 2010;39(5):555-560. http://dx.doi.org/10.1097/MPA.0b013e3181c8b4a5.
39. Kamisawa T, Nakazawa T, Tazuma S, Zen Y, Tanaka A, Ohara H et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis. J Hepatobiliary Pancreat Sci. 2019;26(1):9-42. http://dx.doi.org/10.1002/jhbp.596.