Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2014 Jan;76(1):38-41.
doi: 10.4046/trd.2014.76.1.38. Epub 2014 Jan 29.

A case of immunoglobulin g4-related disease presenting as a pleural mass

Dong Hyun Kim  1 , Kyu Han Koh  1 , Hyeon Sik Oh  2 , Se Joong Kim  3 , Sae Han Kang  1 , Byung Wook Jung  1 , Jun Gyu Song  1 , Mi Ju Cheon  1 , Seon Bin Yoon  1 , Yong Won Park  1 , Young Min Ko  1 , Seung Hyeun Lee  1
Affiliations

A case of immunoglobulin g4-related disease presenting as a pleural mass

Dong Hyun Kim et al. Tuberc Respir Dis (Seoul). 2014 Jan.

Abstract

Immunoglobulin G4 (IgG4)-related disease is a newly recognized condition characterized by fibroinflammatory lesions with dense lymphoplasmacytic infiltration, storiform-type fibrosis and obliterative phlebitis. The pathogenesis is not fully understood but multiple immune-mediated mechanisms are believed to contribute. This rare disease can involve various organs and pleural involvement is even rarer. We report a case of IgG4-related disease involving pleura. A 66-year-old man presented with cough and sputum production for a week. Chest radiography revealed consolidation and a pleural mass at right hemithorax. Treatment with antibiotics resolved the consolidation and respiratory symptoms disappeared, but the pleural mass was unchanged. Video-assisted thoracoscopic surgery was performed. Histopathology revealed dense lymphoplasmacytic infiltration and storiform fibrosis with numerous IgG4-bearing plasma cells. The serum IgG4 level was also elevated. Further examination ruled out the involvement of any other organ. The patient was discharged without further treatment and there is no evidence of recurrence to date.

Keywords: Autoimmune Diseases; Immunoglobulin G; Pleural Neoplasms.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Chest radiography showed a well-defined nodular opacity in right upper hemithorax (arrow) and consolidation at right lower lobe (asterisk).
Figure 2
Figure 2
Chest computed tomography scan showed a consolidative lesion at right lower lobe (A) and a pleura-based mass with contrast enhancement surrounding normal lung parenchyma in right upper lobe (B).
Figure 3
Figure 3
Histopathologic examination showed dense infiltration of inflammatory cells consisting mainly of lymphocytes and plasma cells with some eosinophils (A), and characteristic storiform-type fibrosis (B) (A, B; H&E stain, ×200; inset in A, ×400). In immunohistochemical staining, numerous IgG- and IgG4-positive plasma cells were identified (C, D) (×200).
See this image and copyright information in PMC

References

    1. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–738. - PubMed
    1. Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, et al. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol. 2004;28:1193–1203. - PubMed
    1. Kitagawa S, Zen Y, Harada K, Sasaki M, Sato Y, Minato H, et al. Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner's tumor) Am J Surg Pathol. 2005;29:783–791. - PubMed
    1. Kasashima S, Zen Y, Kawashima A, Konishi K, Sasaki H, Endo M, et al. Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol. 2008;32:197–204. - PubMed
    1. Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–984. - PubMed