A Case of IgG4-Related Lung Disease Mimicking Non-Specific Interstitial Pneumonia.
10.3904/kjm.2015.88.3.308
- Author:
Dong Hui CHO
1
;
Ji Hwan AN
;
Yu Mi KANG
;
Eun Jin CHAE
;
Joon Seon SONG
;
Jin Woo SONG
Author Information
1. Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. jwsong@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
IgG4 related disease;
Lung involvement;
Interstitial lung diseases
- MeSH:
Aged;
Humans;
Immunoglobulin G;
Immunoglobulins;
Inflammation;
Lung Diseases*;
Lung Diseases, Interstitial*;
Male;
Plasma Cells;
Prednisolone;
Thoracic Surgery, Video-Assisted
- From:Korean Journal of Medicine
2015;88(3):308-312
- CountryRepublic of Korea
- Language:Korean
-
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.