Immunoglobulin G4-related thoracic disease: clinical and radiological findings of a Turkish cohort

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Aslı Alkan
Gamze Durhan
Gozde Kubra Yardimci
Umran Ozden Sertcelik
Bayram Farisogullari
Macit Ariyurek
Omer Karadag
Deniz Koksal


IgG4-related disease, Lung, Thorax, Airway, Pleura, Mediastinum


Background and Aim: Thoracic involvement of Immunoglobulin G4-related disease (IgG4-RD) is relatively rare and may be disregarded at the time of initial diagnosis due to its asymptomatic nature. This study aimed to ascertain the prevalence and patterns of thoracic involvement in a retrospective cohort of Turkish patients with IgG4-RD. Methods: A total of 90 patients (47 males and 43 females, with a mean age of 57.7±15.5 years) diagnosed with IgG4-RD were reviewed retrospectively. All computed tomography (CT) scans were re-evaluated by two thoracic radiologists and IgG4-related thoracic disease was assessed on four compartments: The mediastinum, pulmonary parenchyma, airways, and pleura. IgG4-related thoracic disease was categorized as: definite, highly probable, probable or possible. Results: There were 64 patients who had undergone at least one thorax CT examination, and 18 (28%) were diagnosed with IgG4-related thoracic disease. The rate of IgG4-related thoracic disease increased by 20% and reached a ratio of 48.4% (n=31) after a thorough reevaluation of registry data specifically to thoracic findings. The mediastinum was the most frequently involved compartment, affecting 16 (51.6%) patients, followed by pulmonary parenchyma in 14 (45.2%) patients, and airways and pleura in 10 (32.3%) patients each. Other organ involvements were more prevalent and IgG4 levels were higher in patients with thoracic involvement. Eosinophils were significantly elevated in patients with thoracic involvement (p=0.023). Conclusions: IgG4-related thoracic disease is heterogeneous and likely to be more prevalent than currently recognized. The mediastinum is the most frequently involved compartment. It is important to assess IgG4-related thoracic disease at the time of initial diagnosis. Elevated levels of serum IgG4 and eosinophils, as well as a greater number of organ involvements may serve as indicators of thoracic involvement. 

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1. Deshpande V, Zen Y, Chan JK, et al. (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25: 1181-1192.
2. Wallace ZS, Deshpande V, Mattoo H, et al. IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients. Arthritis Rheumatol. 2015 Sep;67(9):2466-75.
3. Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012 Feb;22(1):1-14.
4. Stone JH, Zen Y, Deshpande V. (2012) IgG4-related disease. N Engl J Med 366: 539-551.
5. Ryu JH, Sekiguchi H, Yi ES. Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. Eur Respir J. 2012 Jan;39(1):180-6.
6. Brito-Zerón P, Ramos-Casals M, Bosch X, Stone JH. (2014) The clinical spectrum of IgG4-related disease. Autoimmun Rev 13(12): 1203-1210.
7. Muller R, Habert P, Ebbo M, et al. (2021) Thoracic invovement and imaging patterns in IgG4-related disease. Eur Respir Rev 30(162): 210078.
8. Umehara H, Okazaki K, Masaki Y, et al. (2011) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), Mod Rheumatol 2012; 22(1): 21-30.
9. Deshpande V, Zen Y, Chan J, et al. (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25(9): 1181- 2.
10. Corcoran JP, Culver EL, Anstey RM, et al. (2017) Thoracic involvement in IgG4-related disease in a UK-based patient cohort. Respir Med 132: 117-121.
11. Hansell DM, Bankier AA, MacMahon H, et al. (2008) Fleischner society: glossary of terms for thoracic imaging. Radiology 246: 697-722.
12. Karadağ K, Erden A, Ayhan EA, et al. (2017) The clinical features and outcomes of Turkish patients with IgG4-related disease: a single-center experience.Turk J Med Sci. Nov 13;47(5):1307-1314.
13. Zhang XQ, Chen GP, Wu SC, et al. (2016) Solely lung-involved IgG4-related disease: a case report and review of the literature. Sarcoidosis Vasc Diffuse Lung Dis. 23;33(4):398-406.
14. Muller R, Ebbo M, Habert P, et al. (2023) Thoracic manifestations of IgG4-related disease. Respirology. 28(2):120-31.
15. Sertcelik UO, Oncel A, Koksal D. (2021) Intrathoracic manifestations of immunoglobulin G4-related disease: A pictorial review. Eurasian J Pulmonol 23(2):83-88.
16. Oncel A, Ozden Sertcelik U, Susesi K, et al. (2020) Bronchoscopic Evaluation of Central Airway Involvement in IgG4-related Disease. J Bronchology Interv Pulmonol 27(2):e22-e24.
17. Fujinaga Y, Kadoya M, Kawa S, et al. (2010) Characteristic findings in images of extra-pancreatic lesions associated with autoimmune pancreatitis. Eur J Radiol 76: 228-238.
18. Cheuk W, Yuen HK, Chu SY, Chiu EK, Lam LK, Chan JK. (2008) Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol 32: 671-681.
19. Shrestha B, Sekiguchi H, Colby TV, et al. (2009) Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis: report of 6 and 12 cases with similar histopathology. Am J Surg Pathol 33: 1450- 1462.
20. Carruthers MN, Khosroshahi A, Augustin T, Deshpande V, Stone JH. (2015) The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis 74: 14-18.
21. Maritati F, Rocco R, Accorsi Buttini E, et al. Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients. Front Immunol. 2019 Apr 4;10:693.
22. Morales AT, Cignarella AG, Jabeen IS, et al. (2019) An update on IgG4-related lung disease. Eur J Intern Med. 66: 18-24.
23. Lang D, Zwerina J, Pieringer H. (2016) IgG4-related disease: current challenges and future prospects. Ther Clin Risk Manag. 12: 189-199.
24. Nakajo M, Jinnouchi S, Fukukura Y, et al. (2007) The efficacy of whole-body FDG-PET or PET/CT for autoimmune pancreatitis and associated extrapancreatic autoimmune lesions. Eur J Nucl Med Mol Imaging. 34(12): 2088-2095.
25. Kamisawa T, Anjiki H, Egawa N, Kubota N. Allergic manifestations in autoimmune pancreatitis. Eur J Gastroenterol Hepatol. 2009 Oct;21(10):1136-39.