Isolated IgG4-related mediastinal disease: A case report_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

JIAO Zhenhua ,  YU Jun

Department of Thoracic Surgery, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, 430030, P. R. China;

Corresponding author：

YU Jun, Email: 270384568@qq.com

Keywords：

Immunoglobulin G4-related disease; mediastinum; isolated; surgery; case report

DOI：

10.7507/1007-4848.202304057

Video：

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IgG4-related disease (IgG4-RD) is a chronic, immune-mediated inflammatory and fibrotic condition that can affect virtually any organ system. While some patients experience involvement of only a single organ, isolated IgG4-RD of the mediastinum is even rarer. This article reports a case of a 48-year-old male patient with isolated IgG4-RD of the middle mediastinum. After a biopsy failed to establish a definitive diagnosis, the patient underwent a right thoracotomy-assisted mediastinal tumor resection via video-assisted thoracoscopic surgery. The tumor was completely resected during the procedure, which lasted 130 minutes, with an estimated blood loss of approximately 50 mL. The patient had a favorable postoperative course and satisfactory clinical outcome. No glucocorticoid or other medical treatment was administered postoperatively, and there was no recurrence during a 6-month follow-up period.

1.	Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol, 2012, 25(9): 1181-1192.
2.	Zhang PP, Zhao JZ, Wang M, et al. The clinical characteristics of 346 patients with IgG4-related disease. Zhonghua Nei Ke Za Zhi, 2017, 56(9): 644-649.
3.	Lin W, Lu S, Chen H, et al. Clinical characteristics of immunoglobulin G4-related disease: A prospective study of 118 Chinese patients. Rheumatology (Oxford), 2015, 54(11): 1982-1990.
4.	Umehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol, 2021, 31(3): 529-533.
5.	Wallace ZS, Naden RP, Chari S, 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.
6.	Muller R, Habert P, Ebbo M, et al. Thoracic involvement and imaging patterns in IgG4-related disease. Eur Respir Rev, 2021, 30(162): 210078.
7.	Ichikawa S, Onishi H. Imaging findings of immunoglobulin G4-related disease: From the head to the pelvis. Singapore Med J, 2021, 62(11): 574-581.
8.	Matsui S. IgG4-related respiratory disease. Mod Rheumatol, 2019, 29(2): 251-256.
9.	Yoshikawa R, Igai H, Ohsawa F, et al. Immunoglobulin G4-related disease association with an isolated anterior mediastinal tumor. Ann Thorac Surg, 2019, 107(6): e405-e407.
10.	Kang SR, Kim HR, Nam SJ, et al. Immunoglobulin G4-related disease mimicking an anterior mediastinal tumor. Ann Thorac Surg, 2018, 105(2): e75-e77.
11.	Oda R, Okuda K, Murase T, et al. Thymic inflammatory pseudotumor with multilocular thymic cyst caused by immunoglobulin G4-related disease. Thorac Cancer, 2019, 10(1): 116-119.
12.	Iguchi K, Ishibashi O, Takayashiki N, et al. Mediastinal mass in a patient with IgG4-related disease. Acta Med Int, 2016, 3: 216-218.
13.	Arafat AA, Torky MA, Elhamshary M, et al. Immunoglobulin G4 thymic tumor. Ann Thorac Surg, 2019, 108(4): e229-e231.
14.	Rabadán-Caravaca MD, Ruiz-Carazo E, García-Roa MD. Anterior mediastinal mass associated with febrile syndrome as a single manifestation of IgG4-related disease. Arch Bronconeumol, 2021, 57(11): 702.
15.	Zhang Z, Guan W, Lin Q, et al. Thoracic paravertebral involvement in patients with IgG4-related disease: CT and MR imaging findings. Rheumatology (Oxford), 2020, 59(12): 3878-3885.
16.	Inoue D, Zen Y, Komori T, et al. CT findings of thoracic paravertebral lesions in IgG4-related disease. AJR Am J Roentgenol, 2019, 213(3): W99-W104.
17.	Fu L, Yang B, Xian J, et al. Computed tomograph findings of tissue adjacent to thoracic vertebrae involved by IgG4-related disease. Zhonghua Yi Xue Za Zhi, 2014, 94(41): 3262-3264.
18.	Hino H, Tanaka N, Matsui H, et al. Isolated middle mediastinal mass associated with immunoglobulin G4-related disease. Surg Case Rep, 2021, 7(1): 69.
19.	DeLuzio MR, Rassaei N, Taylor MD, et al. Mediastinal IgG4-related disease manifesting as superior vena cava syndrome. Ann Thorac Surg, 2021, 112(1): e49-e51.
20.	Cha MJ, Chong S, Kim YS, et al. Immunoglobulin G4-related periaortitis involving the aortic arch mimicking a mediastinal tumor. Ann Thorac Surg, 2017, 103(3): e267-e270.
21.	Inoue M, Nose N, Nishikawa H, et al. Successful treatment of sclerosing mediastinitis with a high serum IgG4 level. Gen Thorac Cardiovasc Surg, 2007, 55(10): 431-433.
22.	Villeneuve T, Michaud M, Syrykh C, et al. A silent retrocardiac mass revealing an IgG4-related disease. Respir Med Res, 2021, 79: 100800.
23.	Tang CYL, Chua WM, Cheng LTJ, et al. 18F-FDG PET/CT manifestations of IgG4-related disease. Br J Radiol, 2021, 94(1124): 20210105.
24.	Bai Z, Zhou T, Yu Z, et al. Clinical value of 18F-FDG PET/CT in IgG4-related disease. Ann Nucl Med, 2022, 36(7): 651-660.
25.	Brito-Zerón P, Bosch X, Ramos-Casals M, et al. IgG4-related disease: Advances in the diagnosis and treatment. Best Pract Res Clin Rheumatol, 2016, 30(2): 261-278.
26.	Liu Y, Yang F, Chi X, et al. Needle biopsy compared with surgical biopsy: Pitfalls of small biopsy in histologial diagnosis of IgG4-related disease. Arthritis Res Ther, 2021, 23(1): 54.
27.	Lozano Ros M, Rodríguez Rodríguez ML. Imaging of IgG4-related fibrosing mediastinitis. Radiologia (Engl Ed), 2021, 63(6): 531-533.
28.	Uchida T, Matsubara H, Nagasaka S, et al. Video-assisted thoracoscopic surgery can help enable the complete resection of a mediastinal tumor caused by immunoglobulin G4-related disease and avoid the need for postoperative medication: A case report. Asian J Endosc Surg, 2018, 11(3): 248-251.
29.	Arroyo Pareja L, Triviño A, España C. Tracheal stenosis due to IgG4-related fibrosing mediastinitis. Arch Bronconeumol (Engl Ed), 2019, 55(10): 533.
30.	Khalid M, Weheba I, Abdelsayed A, et al. Superior vena cava stenting in IgG4-associated mediastinal fibrosis. Ann Saudi Med, 2020, 40(2): 155-158.
31.	Khosroshahi A, Wallace ZS, Crowe JL, et al. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol, 2015, 67(7): 1688-1699.
32.	An W, Wu Z, Li M, et al. Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: A retrospective study of 127 Chinese patients. Orphanet J Rare Dis, 2022, 17(1): 307.
33.	Zongfei J, Lingying M, Lijuan Z, et al. Prognostic factors in IgG4-related disease: A long-term monocentric Chinese cohort study. Clin Rheumatol, 2021, 40(6): 2293-2300.
34.	Adam Z, Dastych M, Čermák A, et al. Therapy of immunoglonuline IgG4 related disease (IgG4-RD). Vnitr Lek, 2022, 68(E-6): 15-22.

1. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol, 2012, 25(9): 1181-1192.
2. Zhang PP, Zhao JZ, Wang M, et al. The clinical characteristics of 346 patients with IgG4-related disease. Zhonghua Nei Ke Za Zhi, 2017, 56(9): 644-649.
3. Lin W, Lu S, Chen H, et al. Clinical characteristics of immunoglobulin G4-related disease: A prospective study of 118 Chinese patients. Rheumatology (Oxford), 2015, 54(11): 1982-1990.
4. Umehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol, 2021, 31(3): 529-533.
5. Wallace ZS, Naden RP, Chari S, 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.
6. Muller R, Habert P, Ebbo M, et al. Thoracic involvement and imaging patterns in IgG4-related disease. Eur Respir Rev, 2021, 30(162): 210078.
7. Ichikawa S, Onishi H. Imaging findings of immunoglobulin G4-related disease: From the head to the pelvis. Singapore Med J, 2021, 62(11): 574-581.
8. Matsui S. IgG4-related respiratory disease. Mod Rheumatol, 2019, 29(2): 251-256.
9. Yoshikawa R, Igai H, Ohsawa F, et al. Immunoglobulin G4-related disease association with an isolated anterior mediastinal tumor. Ann Thorac Surg, 2019, 107(6): e405-e407.
10. Kang SR, Kim HR, Nam SJ, et al. Immunoglobulin G4-related disease mimicking an anterior mediastinal tumor. Ann Thorac Surg, 2018, 105(2): e75-e77.
11. Oda R, Okuda K, Murase T, et al. Thymic inflammatory pseudotumor with multilocular thymic cyst caused by immunoglobulin G4-related disease. Thorac Cancer, 2019, 10(1): 116-119.
12. Iguchi K, Ishibashi O, Takayashiki N, et al. Mediastinal mass in a patient with IgG4-related disease. Acta Med Int, 2016, 3: 216-218.
13. Arafat AA, Torky MA, Elhamshary M, et al. Immunoglobulin G4 thymic tumor. Ann Thorac Surg, 2019, 108(4): e229-e231.
14. Rabadán-Caravaca MD, Ruiz-Carazo E, García-Roa MD. Anterior mediastinal mass associated with febrile syndrome as a single manifestation of IgG4-related disease. Arch Bronconeumol, 2021, 57(11): 702.
15. Zhang Z, Guan W, Lin Q, et al. Thoracic paravertebral involvement in patients with IgG4-related disease: CT and MR imaging findings. Rheumatology (Oxford), 2020, 59(12): 3878-3885.
16. Inoue D, Zen Y, Komori T, et al. CT findings of thoracic paravertebral lesions in IgG4-related disease. AJR Am J Roentgenol, 2019, 213(3): W99-W104.
17. Fu L, Yang B, Xian J, et al. Computed tomograph findings of tissue adjacent to thoracic vertebrae involved by IgG4-related disease. Zhonghua Yi Xue Za Zhi, 2014, 94(41): 3262-3264.
18. Hino H, Tanaka N, Matsui H, et al. Isolated middle mediastinal mass associated with immunoglobulin G4-related disease. Surg Case Rep, 2021, 7(1): 69.
19. DeLuzio MR, Rassaei N, Taylor MD, et al. Mediastinal IgG4-related disease manifesting as superior vena cava syndrome. Ann Thorac Surg, 2021, 112(1): e49-e51.
20. Cha MJ, Chong S, Kim YS, et al. Immunoglobulin G4-related periaortitis involving the aortic arch mimicking a mediastinal tumor. Ann Thorac Surg, 2017, 103(3): e267-e270.
21. Inoue M, Nose N, Nishikawa H, et al. Successful treatment of sclerosing mediastinitis with a high serum IgG4 level. Gen Thorac Cardiovasc Surg, 2007, 55(10): 431-433.
22. Villeneuve T, Michaud M, Syrykh C, et al. A silent retrocardiac mass revealing an IgG4-related disease. Respir Med Res, 2021, 79: 100800.
23. Tang CYL, Chua WM, Cheng LTJ, et al. 18F-FDG PET/CT manifestations of IgG4-related disease. Br J Radiol, 2021, 94(1124): 20210105.
24. Bai Z, Zhou T, Yu Z, et al. Clinical value of 18F-FDG PET/CT in IgG4-related disease. Ann Nucl Med, 2022, 36(7): 651-660.
25. Brito-Zerón P, Bosch X, Ramos-Casals M, et al. IgG4-related disease: Advances in the diagnosis and treatment. Best Pract Res Clin Rheumatol, 2016, 30(2): 261-278.
26. Liu Y, Yang F, Chi X, et al. Needle biopsy compared with surgical biopsy: Pitfalls of small biopsy in histologial diagnosis of IgG4-related disease. Arthritis Res Ther, 2021, 23(1): 54.
27. Lozano Ros M, Rodríguez Rodríguez ML. Imaging of IgG4-related fibrosing mediastinitis. Radiologia (Engl Ed), 2021, 63(6): 531-533.
28. Uchida T, Matsubara H, Nagasaka S, et al. Video-assisted thoracoscopic surgery can help enable the complete resection of a mediastinal tumor caused by immunoglobulin G4-related disease and avoid the need for postoperative medication: A case report. Asian J Endosc Surg, 2018, 11(3): 248-251.
29. Arroyo Pareja L, Triviño A, España C. Tracheal stenosis due to IgG4-related fibrosing mediastinitis. Arch Bronconeumol (Engl Ed), 2019, 55(10): 533.
30. Khalid M, Weheba I, Abdelsayed A, et al. Superior vena cava stenting in IgG4-associated mediastinal fibrosis. Ann Saudi Med, 2020, 40(2): 155-158.
31. Khosroshahi A, Wallace ZS, Crowe JL, et al. International consensus guidance statement on the management and treatment of IgG4-related disease. Arthritis Rheumatol, 2015, 67(7): 1688-1699.
32. An W, Wu Z, Li M, et al. Clinical characteristics and therapeutic response of immunoglobulin G4-related disease: A retrospective study of 127 Chinese patients. Orphanet J Rare Dis, 2022, 17(1): 307.
33. Zongfei J, Lingying M, Lijuan Z, et al. Prognostic factors in IgG4-related disease: A long-term monocentric Chinese cohort study. Clin Rheumatol, 2021, 40(6): 2293-2300.
34. Adam Z, Dastych M, Čermák A, et al. Therapy of immunoglonuline IgG4 related disease (IgG4-RD). Vnitr Lek, 2022, 68(E-6): 15-22.

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