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KMID : 0811820120160020132
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Journal of Korean Society of Pediatric Nephrology
2012 Volume.16 No. 2 p.132 ~ p.137
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A Case of Microscopic Polyangiitis Initially Suspected with Henoch-Schonlein Purpura Nephritis
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Im Jing-Geun
Moon Kyung-Chul
Koo Ja-Wook
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Abstract
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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-Schonlein purpura nephritis such as purpuric skin rash, abdominal pain, arthralgia on both knees, massive proteinuria and microscopic hematuria. So initially we suspected Henoch-Schonlein 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-Schonlein purpura nephritis.
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KEYWORD
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Microscopic polyangiitis, p-ANCA, Vasculitis, Henoch-Schonlein purpura
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