Tuberc Respir Dis.  2014 Jan;76(1):42-45.

A Case Report of IgG4-Related Disease Clinically Mimicking Pleural Mesothelioma

• Choi IH
• Jang SH
• Lee S
• Han J
• Kim TS
• Chung MP

Abstract

An immunoglobulin G4 (IgG4)-related disease is a recently emerging entity, and a few cases of IgG4-related disease in lung and pleura have been reported. Herein, we report the case of a 74-year-old man with IgG4-related disease of lung and pleura, clinically suspicious of malignant mesothelioma. Chest computed tomography showed diffuse nodular pleural thickening, and microscopic finding disclosed diffuse thickening of visceral pleura with infiltrations of many lymphoplasma cells with increased number of IgG4-positive plasma cells and a few multinucleated giant cells. It is important for pathologists and clinicians to recognize this rare entity and its histologic finding, because it can be confused with malignant tumors on the radiologic examination although it can be treated with steroid therapy.

Keyword

Lung Diseases; Pleural Diseases; Immunoglobulin G; Plasma Cells

MeSH Terms

Aged
Giant Cells
Humans
Immunoglobulin G
Immunoglobulins
Lung
Lung Diseases
Mesothelioma*
Plasma Cells
Pleura
Pleural Diseases
Thorax
Immunoglobulin G
Immunoglobulins

Figure

• 

  Figure 1 Radiologic findings of IgG4-related disease of lung and pleura. (A, C) Chest X-ray (taken 1 year ago) and computed tomography (taken 6 months ago) demonstrate pleural effusion with patchy ground-glass opacity lesions in the right lung. (D) Subsequent computed tomography reveals newly developed, diffuse patchy lesions with pleural thickening and fissural nodularity in both lung, which radiologically suggests mesothelioma or pleural seeding from the lung cancer. (B) These lesions show hot uptake on positron emission tomography. (E) After steroid therapy, the lesions of both lobes are improved with residual small nodule in the left pleura.

• 

  Figure 2 Microscopic findings of IgG4-related disease of lung and pleura (A) Scanned view of the lesion displays pleural thickening and subpleural fibrosis. (B-D, F, G) Infiltrate cells are mainly composed of lymphocytes and plasma cells in the background of dense fibrosis. Dispersed giant cells are also noted in some areas (C, F, and G, H&E stain; B and D, Elastic stain; B, ×12.5; C and D, ×200; E, ×100; F, ×40; G, ×400). (E) Small blood vessels are infiltrated by lymphoplasma cells without fibrinoid necrosis or granuloma formation. Immunohistochemical stainings for IgG (H) and IgG4 (I) reveal increased ratio of IgG4/IgG positive plasma cells, estimated at about 40% (H and I, ×200).

Reference

1. Zen Y, Inoue D, Kitao A, Onodera M, Abo H, Miyayama S, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009; 33:1886–1893.

2. Divatia M, Kim SA, Ro JY. IgG4-related sclerosing disease, an emerging entity: a review of a multi-system disease. Yonsei Med J. 2012; 53:15–34.

3. Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010; 17:303–332.

4. Shigemitsu H, Koss MN. IgG4-related interstitial lung disease: a new and evolving concept. Curr Opin Pulm Med. 2009; 15:513–516.

5. Yamashita K, Haga H, Kobashi Y, Miyagawa-Hayashino A, Yoshizawa A, Manabe T. Lung involvement in IgG4-related lymphoplasmacytic vasculitis and interstitial fibrosis: report of 3 cases and review of the literature. Am J Surg Pathol. 2008; 32:1620–1626.

6. Park HY, Han J, Kang G, Yi CA, Chung MP. IgG4-related lung disease presenting as a consolidative mass: a case report. J Lung Cancer. 2010; 9:103–105.

7. Yoo JW, Roh JH, Lim CM, Lee SD, Kim WS, Kim DS, et al. Two cases of pulmonary involvement of immunoglobulin G4 related autoimmune disease. Tuberc Respir Dis. 2009; 67:359–363.

8. Koss M, Travis W, Moran C, Hochholzer L. Pseudomesotheliomatous adenocarcinoma: a reappraisal. Semin Diagn Pathol. 1992; 9:117–123.

9. Kim EY, Kim TS, Han J, Choi JY, Kwon OJ, Kim J. Thoracic epithelioid hemangioendothelioma: imaging and pathologic features. Acta Radiol. 2011; 52:161–166.

10. Zen Y, Fujii T, Sato Y, Masuda S, Nakanuma Y. Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease. Mod Pathol. 2007; 20:884–894.

11. Yamamoto H, Yamaguchi H, Aishima S, Oda Y, Kohashi K, Oshiro Y, et al. Inflammatory myofibroblastic tumor versus IgG4-related sclerosing disease and inflammatory pseudotumor: a comparative clinicopathologic study. Am J Surg Pathol. 2009; 33:1330–1340.