Tuberc Respir Dis.  2014 Jan;76(1):38-41.

A Case of Immunoglobulin G4-Related Disease Presenting as a Pleural Mass

• Kim DH
• Koh KH
• Oh HS
• Kim SJ
• Kang SH
• Jung BW
• Song JG
• Cheon MJ
• Yoon SB
• Park YW
• Ko YM
• Lee SH

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.

Keyword

Immunoglobulin G; Autoimmune Diseases; Pleural Neoplasms

MeSH Terms

Aged
Anti-Bacterial Agents
Autoimmune Diseases
Cough
Fibrosis
Humans
Immunoglobulin G
Immunoglobulins*
Phlebitis
Plasma Cells
Pleura
Pleural Neoplasms
Radiography
Rare Diseases
Recurrence
Sputum
Thoracic Surgery, Video-Assisted
Thorax
Anti-Bacterial Agents
Immunoglobulin G
Immunoglobulins

Figure

• 

  Figure 1 Chest radiography showed a well-defined nodular opacity in right upper hemithorax (arrow) and consolidation at right lower lobe (asterisk).

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  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).

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  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).

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