Korean J Med.
2015 Mar;88(3):308-312. 10.3904/kjm.2015.88.3.308.
A Case of IgG4-Related Lung Disease Mimicking Non-Specific Interstitial Pneumonia
- Affiliations
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- 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. jwsong@amc.seoul.kr
- 2Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
- 3Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
- KMID: 2082592
- DOI: http://doi.org/10.3904/kjm.2015.88.3.308
Abstract
- Immunoglobulin (Ig) G4-related disease is a recently described systemic inflammatory disease characterized by high serum IgG4 concentrations and sclerosing inflammation of numerous IgG4-positive plasma cells that responds favorably to steroid treatment. Although initial description of this disorder focused on its pancreatic presentation, it has become apparent that it is a systemic disease. In this report, we describe a case of IgG4-related lung disease presenting as non-specific interstitial pneumonia in a 78-year-old male with interstitial lung disease. Pathological examination through video-assisted thoracic surgery showed a non-specific interstitial pneumonia pattern and numerous (> 50/high-power field) infiltrating IgG4-positive plasma cells. Laboratory tests also revealed a high serum IgG4 concentration. Prednisolone therapy was initiated and his symptoms and reticular opacity improved after two months of treatment.
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