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막성사구체신염과 동반된 폐포단백증 1예

Concurrence of Membranous Glomerulonephritis and Pulmonary Alveolar Proteinosis

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Membranous glomerulonephritis is one of the most common causes of nephrotic syndrome in adults. Pulmonary alveolarproteinosis (PAP) is a rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli. We describe a61-year-old man with concurrent membranous glomerulonephritis and PAP, which is very rare; both are pathophysiologicallyrelated to an abnormal immune response. A patient came to hospital with leg edema but no respiratory symptoms. Chest X-ray andCT showed classical PAP findings, which are ground-glass opacities with interlobular septal thickening, in both lung fields. Abubbly whitish secretion retrieved via broncho-alveolar lavage showed neutrophils and lymphocytes as well as Periodicacid-Schiff-positive proteinaceous materials. A kidney biopsy revealed findings of membranous glomerulonephritis with irregularsubepithelial deposits by electron microscopy. At 1 year after diagnosis, the membranous glomerulonephritis was well undercontrol with steroids and mycophenolate mofetil but PAP became aggravated gradually and whole-lung lavage was needed.

Abstract

Membranous glomerulonephritis is one of the most common causes of nephrotic syndrome in adults. Pulmonary alveolarproteinosis (PAP) is a rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli. We describe a61-year-old man with concurrent membranous glomerulonephritis and PAP, which is very rare; both are pathophysiologicallyrelated to an abnormal immune response. A patient came to hospital with leg edema but no respiratory symptoms. Chest X-ray andCT showed classical PAP findings, which are ground-glass opacities with interlobular septal thickening, in both lung fields. Abubbly whitish secretion retrieved via broncho-alveolar lavage showed neutrophils and lymphocytes as well as Periodicacid-Schiff-positive proteinaceous materials. A kidney biopsy revealed findings of membranous glomerulonephritis with irregularsubepithelial deposits by electron microscopy. At 1 year after diagnosis, the membranous glomerulonephritis was well undercontrol with steroids and mycophenolate mofetil but PAP became aggravated gradually and whole-lung lavage was needed.

참고문헌 (7)

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  7. Tazawa R, "Inhaled granulocyte", macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis, 2010, pp.181, 1345~1354

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