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Korean J Med > Volume 88(3); 2015 > Article
The Korean Journal of Medicine 2015;88(3): 308-312.
비특이적 간질성 폐렴의 양상으로 발생한 IgG4 연관 폐 질환의 1예
조동희1, 안지환1, 강유미1, 채은진2, 송준선3, 송진우1
1울산대학교 의과대학 서울아산병원 내과
2울산대학교 의과대학 서울아산병원 영상의학과
3울산대학교 의과대학 서울아산병원 병리과
A Case of IgG4-Related Lung Disease Mimicking Non-Specific Interstitial Pneumonia
Dong Hui Cho1, Ji Hwan An1, Yu Mi Kang1, Eun Jin Chae2, Joon Seon Song3, Jin Woo Song1
1Departments of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Departments of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
3Departments of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Jin Woo Song ,Tel: +82-2-3010-3993, Fax: +82-2-3010-6968, Email: jwsong@amc.seoul.kr
Received: May 13, 2014;   Revised: July 22, 2014;   Accepted: August 5, 2014.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords: IgG4 related disease; Lung involvement; Interstitial lung diseases
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