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Journal of the Korean Society of Pediatric Nephrology 2012;16(2): 132-137. doi: https://doi.org/10.3339/jkspn.2012.16.2.132
Henoch-Sch$ddot{o}$nlein 자반병 신염으로 의심했던 현미경적 다발혈관염 1례
임종근, 문경철, 구자욱
1인제대학교 상계백병원 소아청소년과
2서울대학교 의과대학 병리학교실
3인제대학교 상계백병원 소아청소년과
A Case of Microscopic Polyangiitis Initially Suspected with Henoch-Sch$ddot{o}$nlein Purpura Nephritis
Jong Geun Im, Kyung Chul Moon, Ja Wook Koo
1Department of Pediatrics, Sanggye Paik Hospital, Inje University College of Medicine
2Department of Pathology, Seoul National University College of Medicine
3Department of Pediatrics, Sanggye Paik Hospital, Inje University College of Medicine
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ABSTRACT
Microscopic polyangiitis (MPA) is systemic small vessel vasculitis that is very rare in childhood. MPA is characterized by pauci-immune necrotizing small vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Approximately 90% of patients have glomerulonephritis that is accompanied by a variety of other organ involvement. A 10-year-old girl visited our clinic with clinical manifestations suggestive Henoch-Sch$ddot{o}$nlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Sch$ddot{o}$nlein purpura nephritis. However, later her perinuclear-antineutrophil cytoplasmic antibodies(p-ANCA) test was positive, and her renal biopsy was consistent with microscopic polyangiitis. We began steroid therapy, combined with cyclophosphamide, ACE inhibitor. Currently she is a 12-year old, and until now she has been regularly examined in the outpatient. We report a case of microscopic polyangiitis initially suspected with Henoch-Sch$ddot{o}$nlein purpura nephritis.
Key words: Microscopic polyangiitis | p-ANCA | Vasculitis | Henoch-Sch$\ddot{o}$nlein purpura
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