Abstract
We report a rare case of a IgG4-related disease presenting with recurrent pleural effusion, pleural thickness and multiple mediastinal lymphadenopathies and no involvement of other extrathoracic organs. A 65-year-old man with a previous asbestos exposure presented with cough and pain discomfort. A large right pleural effusion was detected and evacuated (siero-haematic liquid). With the suspicious of a pleural mesothelioma, a CT-scan before and a 18F-FDG PET/CT-scan later were performed revealing multiple pleural thickenings and multiple mediastinal lymphadenopathies with radiotracer uptake. EBUS-TBNA EBUS-TBNA did not result in a formal pathological diagnosis; thus, multiple pleural biopsy were performed via right thoracoscopy. At pathology the pleura was markedly thickened by a chronic fibroinflammatory process with scattered lymphoid follicles and a large number of mature plasma cells. Immunohistochemistry shows a mixed B (CD20+) and T (CD3+) population of lymphocytes, without light chain restriction and an increased number of IgG4-positive plasma cells. A presumptive diagnosis of IgG4-related disease was formulated. Total body CT-scan excluded other organ involvement. Blood test showed elevated serum IgG4 concentrations (253 mg/dL) and mild elevation of acute-phase reactants (C-reactive protein 10.7 mg/L). Autoimmune profile was negative. A diagnosis of definite IgG4-related disease was made, and treatment with prednisone 50 mg/day was started.
Similar content being viewed by others
References
Umehara H, Okazaki K, Masaki Y et al (2012) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 22(1):21–30
Wang A, Fan J, Chen X, Wang S (2018) An initial exploration for comprehensive assessment of IgG4-related lung disease: analyses on the cases enrolled from a systematic review. J Thorac Dis 10(3):1825–1841. https://doi.org/10.21037/jtd.2018.01.149
Fei Y, Shi J, Lin W, Chen Y, Feng R, Wu Q, Gao X, Xu W, Zhang W, Zhang X, Zhao Y, Zeng X, Zhang F (2015) Intrathoracic involvements of immunoglobulin G4-related sclerosing disease. Medicine (Baltimore) 94(50):e2150. https://doi.org/10.1097/MD.0000000000002150
Corcoran JP, Culver EL, Anstey RM, Talwar A, Manganis CD, Cargill TN, Hallifax RJ, Psallidas I, Rahman NM, Barnes E (2017) Thoracic involvement in IgG4-related disease in a UK-based patient cohort. Respir Med 132:117–121. https://doi.org/10.1016/j.rmed.2017.10.005 (Epub 2017 Oct 10)
Kita T, Araya T, Ichikawa Y, Terada N, Kawashima A, Kasashima S, Kasahara K (2018) IgG4-related pleuritis with no other organ involvement. Am J Med Sci 356(5):487–491. https://doi.org/10.1016/j.amjms.2018.05.004 (Epub 2018 May 9)
Kato E, Takayanagi N, Ishiguro T, Kagiyama N, Shimizu Y, Sugita Y (2014) IgG4-related pleuritis with chylothorax. Intern Med 53(14):1545–1548 (Epub 2014 Jul 15)
Kitada M, Matuda Y, Hayashi S, Ishibashi K, Oikawa K, Miyokawa N, Ohsaki Y (2013) IgG4-related lung disease showing high standardized uptake values on FDG-PET: report of two cases. J Cardiothorac Surg 25(8):160. https://doi.org/10.1186/1749-8090-8-160
Kamisawa T, Zen Y, Pillai S, Stone JH (2015) IgG4-related disease. Lancet 385(9976):1460–1471
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
None.
Ethical Approval
The manuscript was written in compliance with ethical standards.
Informed Consent
A written informed consent has been obtained before publication.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Lococo, F., Di Stefano, T., Rapicetta, C. et al. Thoracic Hyper-IgG4-Related Disease Mimicking Malignant Pleural Mesothelioma. Lung 197, 387–390 (2019). https://doi.org/10.1007/s00408-019-00224-5
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00408-019-00224-5