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Journal of the Korean Society of Pediatric Nephrology 2005;9(2): 231-236.
소아기 류마티스 관절염에서 발견된 ANCA 연관 극소면역성 반월상 사구체신염 1례
황유식, 이영준, 안선영, 김동수, 이재승, 정현주
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 소아과학교실
3연세대학교 의과대학 소아과학교실
4연세대학교 의과대학 소아과학교실
5연세대학교 의과대학 소아과학교실
6연세대학교 의과대학 병리학교실
A Case of ANCA-associated Pauci-immune Crescentic Glomerulonephritis in Juvenile Rheumatoid Arthritis
You Sik Hwang, Young Jun Rhie, Sun Young Ahn, Dong Soo Kim, Jae Seung Lee, Hyun Joo Jeong
1Department of Pediatrics, The Institute of Kidney Disease, Yonsei University, College of Medicine
2Department of Pediatrics, The Institute of Kidney Disease, Yonsei University, College of Medicine
3Department of Pediatrics, The Institute of Kidney Disease, Yonsei University, College of Medicine
4Department of Pediatrics, The Institute of Kidney Disease, Yonsei University, College of Medicine
5Department of Pediatrics, The Institute of Kidney Disease, Yonsei University, College of Medicine
6Department of Pathology, Yonsei University, College of Medicine
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ABSTRACT
Juvenile rheumatoid arthritis(JRA) is the most common major connective tissue disease in children. Renal involvement in JRA is rare. Among the renal lesions that have been reported in JRA, amyloidosis and drug-induced nephropathy are the most common. Crescentic glomerulonephritis in JRA has rarely been reported. We report a case of ANCA-associated pauci-immune crescentic glomerulonephritis in JRA. The patient was a 15-year old boy with a 3-year history of JRA. He presented with gross hematuria, proteinuria, positive p-ANCA and elevation of BUN and creatinine. Pathologic findings revealed focal necrotizing and crescentic glomerulonephritis. There were no significant immunoglobulin or complement deposits. His renal function recovered after intravenous methylprednisolone pulse therapy and oral steroid use. In Korea, this is the first reported case of pauci-immune crescentic glomerulonephritis in JRA. (J Korean Soc Pediatr Nephrol 2005;9:231-236)
Key words: Juvenile rheumatoid arthritis | ANCA | Crescentic glomerulonephritis
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