Tuberc Respir Dis.  2014 Apr;76(4):179-183.

A Case of IgG4-Related Disease Presenting as Massive Pleural Effusion and Thrombophlebitis

• Choi JH
• Sim JK
• Oh JY
• Lee EJ
• Hur GY
• Lee SH
• Lee SY
• Kim JH
• Lee SY
• Shin C
• Shim JJ
• In KH
• Kang KH
• Min KH

Abstract

Immunoglobulin (Ig) G4-related disease is a recently recognized systemic fibroinflammatory condition characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with elevated circulating levels of IgG4. The disease can either be localized to one or two organs, or present as diffuse multi-organ disease. Furthermore, lesions in different organs can present simultaneously or metachronously. In the pulmonary manefestations, lesions associated with IgG4-related disease have been described in the lung parenchyma, airways and pleura, as well as the mediastinum. We report a case of IgG4-related disease presenting as massive pleural effusion and thrombophlebitis.

Keyword

Immunoglobulin G; Pleural Effusion; Thrombophlebitis

MeSH Terms

Immunoglobulin G
Immunoglobulins
Lung
Mediastinum
Plasma Cells
Pleura
Pleural Effusion*
Thrombophlebitis*
Immunoglobulin G
Immunoglobulins

Figure

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  Figure 1 (A-D) Imaging study on admission day showed thrombophlebitis of neck vein and enlarged lymph node in the left neck and axillar area with both pleural effusion and ascites and diffuse soft tissue edema. (A) Posterior-anterior (PA) view of chest X-ray. (B-D) Transverse section of chest computed tomography (CT). (E) Coronal section of chest CT. (F-H) Imaging study at 2 months after systemic steroid and anticoagulation showed markedly improved previous imaging finding. (F) PA view of chest X-ray. (G-I) Transverse section of chest CT. (J) Coronal section of chest CT.

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  Figure 2 Neck computed tomography on admission day showed showed thrombophlebitis of neck vein and enlarged lymph node at left neck and axillar area. (A) Transverse section. (B) Coronal section.

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  Figure 3 (A-E) Pleural biopsy showed chronic inflammation with lymphoplasmacytic infiltration and fibrosis (A, H&E stain, ×40; B, H&E stain, ×200; C, H&E stain, ×400; D, immunohistochemical stain for IgG, ×400; E, immunohistochemical stain for IgG4, ×400). (F-I) Lymph node biopsy of neck on previous admission also showed chronic inflammation with lymphoplasmacytic infiltration (F, H&E stain, ×40; G, H&E stain, ×400; H, immunohistochemical stain for IgG, ×400; I, immunohistochemical stain for IgG4, ×400).

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