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Case Report
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Korean J Med. 2015;88(3):308-312. Published online March 1, 2015.
DOI: https://doi.org/10.3904/kjm.2015.88.3.308
- 비특이적 간질성 폐렴의 양상으로 발생한 IgG4 연관 폐 질환의 1예
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조동희1, 안지환1, 강유미1, 채은진2, 송준선3, 송진우1
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1울산대학교 의과대학 서울아산병원 내과
2울산대학교 의과대학 서울아산병원 영상의학과
3울산대학교 의과대학 서울아산병원 병리과
- A Case of IgG4-Related Lung Disease Mimicking Non-Specific Interstitial Pneumonia
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Dong Hui Cho1, Ji Hwan An1, Yu Mi Kang1, Eun Jin Chae2, Joon Seon Song3, Jin Woo Song1
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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.
- 특발성 비특이적 간질성 폐 질환과 IgG4 related lung disease는 증상, 폐기능 검사, 영상의학 소견만으로는 감별이 어려운 질환이다. 하지만 IgG4 related lung disease는 steroid 치료에 좋은 치료 반응을 보이는 것으로 알려져 있어 이들의 감별은 매우 중요하다고 할 수 있다. 본 증례는 특발성 간질성 폐 질환, 특히 비특이적 간질성 폐렴에서 혈청 IgG4의 증가 혹은 조직 소견상 lymphoplasmacytic infiltration이 다수 관찰되고 obliterative phlebitis, storiform fibrosis 등이 관찰된다면 IgG4 연관 질환의 폐 침범 가능성을 고려해야 함을 시사해 준다.
߽ɾ :IgG4 연관 질환; 폐 침범; 간질성 폐 질환
- 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.
Keywords :IgG4 related disease, Lung involvement, Interstitial lung diseases
