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KMID : 0882420150880030308
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Korean Journal of Medicine
2015 Volume.88 No. 3 p.308 ~ p.312
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A Case of IgG4-Related Lung Disease Mimicking Non-Specific Interstitial Pneumonia
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Cho Dong-Hui
Ahn Ji-Hwan
Kang Yu-Mi
Chae Eun-Jin
Song Joon-Seon
Song Jin-Woo
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Abstract
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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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KEYWORD
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IgG4 related disease, Lung involvement, Interstitial lung diseases
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