Tuberc Respir Dis.  2015 Oct;78(4):396-400. 10.4046/trd.2015.78.4.396.

A Case of Extensive IgG4-Related Disease Presenting as Massive Pleural Effusion, Mediastinal Mass, and Mesenteric Lymphadenopathy in a 16-Year-Old Male

• Goag EK
• Park JE
• Lee EH
• Park YM
• Kim CY
• Lee JM
• Kim YJ
• Kim YS
• Kim SK
• Chang J
• Park MS
• Chung KS

• KMID: 2320714
• DOI: http://doi.org/10.4046/trd.2015.78.4.396

Abstract

IgG4-related disease is an immune-mediated fibro-inflammatory disease, characterized by lymphoplasmacytic infiltration composed of IgG4-positive plasma cells of various organs with elevated circulating levels of IgG4. This disease is now reported with increasing frequency and usually affects middle-aged men. Massive pleural effusion in children is an uncommon feature in IgG4-related disease. Here, we report a case of a 16-year-old male patient with extensive IgG4-related disease presenting with massive pleural effusion, mediastinal mass, and mesenteric lymphadenopathy.

Keyword

Pleural Effusion; Mediastinum

MeSH Terms

Adolescent*
Child
Humans
Immunoglobulin G
Lymphatic Diseases*
Male*
Mediastinum
Plasma Cells
Pleural Effusion*
Immunoglobulin G

Figure

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  Figure 1 (A-D) Imaging study on admission day showing massive bilateral pleural effusion with loculation, interlobular septal thickening, bronchial wall thickening, and soft tissue density at the anterior mediastinum. (E) A chest X-ray in the posterior-anterior view after 4 months of treatment with systemic steroids and azathioprine showing markedly decreased bilateral pleural effusion. RT-PA, right posterior-anterior view.

• 

  Figure 2 (A-F) Mediastinal biopsy showing papillary proliferation of reactive mesothelial cells and dense collagenous fibrosis with stromal dense lymphoplasmacytic infiltration (IgG4-/IgG-positive plasma cell, >40%; IgG4, maximal count 62/high-power field; human melanoma black 45, negative) (A, H&E stain, ×12.5; B and C, H&E stain, ×40; D, H&E stain, ×100; E, IgG immunostaining, ×200; F, IgG4 immunostaining, ×200).

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