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Pleural effusion related to IgG4.
Yoriyuki Murata1,2, Keisuke Aoe1,2, Yusuke Mimura1
1The Department of Clinical Research.
Current Opinion in Pulmonary Medicine
|March 19, 2019
Summary
Immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized as a cause of unexplained exudative pleural effusions. Diagnosis involves identifying IgG4-positive plasma cells in pleural biopsies, and treatment typically involves steroids.
Area of Science:
- Pulmonology
- Immunology
- Rheumatology
Background:
- Exudative pleural effusions often have unclear causes despite thorough investigation.
- Immunoglobulin G4-related disease (IgG4-RD) is a newly identified fibroinflammatory condition affecting multiple organs, including the lungs.
- This review focuses on the role of IgG4 in exudative pleural effusions of unknown etiology.
Purpose of the Study:
- To review the involvement of IgG4 in exudative pleural effusions where the cause remains undetermined.
- To highlight IgG4-related disease as a potential diagnosis for unexplained pleural effusions.
Main Methods:
- Review of current literature on IgG4-related disease and pleural effusions.
- Analysis of diagnostic criteria and histological findings in IgG4-related pleural lesions.
- Discussion of the pathogenesis and treatment of IgG4-related pleural involvement.
Main Results:
- Immunoglobulin G4 is implicated in a subset of patients with undiagnosed pleural effusions.
- Pleural manifestations of IgG4-RD can occur independently or with other organ involvement.
- Histological hallmarks include lymphoplasmacytic infiltrate with abundant IgG4-positive plasma cells in the pleura.
Conclusions:
- Consider IgG4-related pleural lesions in patients with unexplained pleural effusion and lymphoplasmacytic infiltration on biopsy.
- Pleural effusions in IgG4-RD are often associated with pleural thickening.
- Systemic corticosteroid therapy is an effective treatment for IgG4-related pleural disease.
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