혼합결합조직병에 동반된 최소변화콩팥병증 1예
Minimal Change in Nephrotic Syndrome Associated with Mixed Connective Tissue Disease
윤정아, 장문, 장재원, 이은경,
소속 상세정보
윤정아 ( Yoon Jung-A )
University of Ulsan College of Medicine Asan Medical Center Department of Internal Medicine Division of Nephrology
장문 ( Jang Mun )
Yemidam Hospital Department of Internal Medicine
장재원 ( Chang Jai-Won )
University of Ulsan College of Medicine Asan Medical Center Department of Internal Medicine Division of Nephrology
이은경 ( Lee Eun-Kyoung )
Dankook University College of Medicine Dankook University Hospital Department of Neurology
Abstract
Mixed connective tissue disease (MCTD) is a rheumatologic disorder that has combined features of systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis with the anti-U1 ribonucleoprotein (RNP) antibody. Although renal involvement is not included in any of the diagnostic criteria, it has been observed in 20% of MCTD patients. The most common renal disease is immune complex nephropathy, histologically classified as membranous glomerulonephritis, but minimal change disease (MCD) is very rare and has only been reported once previously. A previously healthy 33-year-old woman presented with generalized edema, puffy hands, arthralgia, and Raynaud’s phenomenon. We diagnosed MCD with proteinuria (> 9 g/day) and hypoalbuminemia (< 1.0 g/dL) by renal biopsy, and MCTD with a simultaneously high titer of the anti-U1 RNP antibody. The patient was treated with 200 mg/day cyclosporine and 30 mg/day prednisolone. The proteinuria and hypoalbuminemia normalized 2 months later. We report a patient with severe MCD associated with MCTD.
키워드
혼합결합조직병; 최소변화콩팥병증; 신증후군
Mixed connective tissue disease; Nephrosis, lipoid; Nephrotic syndrome
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