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논문분류	춘계학술대회 초록집
제목	Fibrillary Glomerulonephritis Associated with Monoclonal Gammopathy of Undetermined Significance
저자	Jong Man Park1, Ji Yeon Shin1, Nari Shin2, Sang Heon Song1, Harin Rhee1, Woo Jin Jung1, Su Min Park1, Jin Suk Kang1, Nakyoung Hwang1, Il Young Kim1, Dong Won Lee1, Soo Bong Lee1, Eun Young Seong1, Ihm Soo Kwak1
출판정보	2015; 2015(1):
키워드	원섬유성 사구체신염,MGUS
초록	Fibrillary glomerulonephritis (FGN) is a rare disease diagnosed by deposition of fibrillary material in glomerulus. Generally FGN was associated with conditions of immune system dysfunction or chronic immune system activation. We report the case of 70-yr-old woman with a diagnosis of FGN. She presented to the outpatient clinic for general edema and the initial blood lab showed hypoalbuminemia, nephrotic level of proteinuria and dyslipidemia. She admitted to evaluate nephrotic syndrome and to manage general edema and hypertension. A renal biopsy was performed to evaluate the cause of nephrotic syndrome. Light microscopy study showed lobular eccentuation pattern with severe endocapillary, mild mesangial hypercellularity and extracapillary hypercellularity. On immunofluorescence study, IgG, IgA, Kappa and Lambda were positive on glomerular capillary wall and mesangium. On electron microscopy study, variable fibrilary deposits (12-17nm) were identified on the mesangium, subendothelium, and subepithelium. Congo red stain was negative. Fibrillary Glomerulonephritis was diagnosed. Electoporesis and immunofixation study showed monoclonal band in lambda region. The aspirate smear of the bone marrow showed infiltration of increased plasma cells (8% among total nucleated cells). Monoclonal gammopathy of undetermined significance (MGUS) was confirmed. For FGN combined with MGUS, treatment was started with intravenous methylprednisolone and followed by oral prednisolone. She was followed in the outpatient clinic. To our knowledge, there has not been reported FGN combined with MGUS in Korea and only several cases were reported in the English literature. This report showed a rare case of FGN with MGUS.
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