Childhood microscopic polyangiitis associated with MPO-ANCA

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• 作者：Amira Peco-Antic ; Branka Bonaci-Nikolic ; Gordana Basta-Jovanovic ; Mirjana Kostic ; Jasmina Markovic-Lipkovski ; Milos Nikolic and Brankica Spasojevic
• 关键词：Acute renal failure ; Alpha SMA ; ANCA ; Childhood ; Microscopic polyangiitis
• 刊名：Pediatric Nephrology
• 出版年：2006
• 出版时间：January 2006
• 年：2006
• 卷：21
• 期：1
• 页码：46-53
• 全文大小：236 KB

文摘

We reviewed the clinical, histological and serological parameters of microscopic polyangiitis (MPA) associated with antineutrophil cytoplasmic antibodies (ANCA) specific to myeloperoxidase (MPO). Six girls and one boy aged 12.0±2.6 years (7–15 years) met the following inclusion criteria: (1) clinical manifestations of systemic small vessel involvement; (2) histological demonstration of pauci-immune necrotizing glomerulonephritis; and (3) serological findings of increased concentration of MPO-ANCA by ELISA test. The main clinical manifestations were: influenza-like symptoms (100 % ), hematuria/proteinuria (100 % ), purpura (100 % ), pulmonary-renal syndrome (57 % ), acute renal failure (ARF) (29 % ), ischemic cerebral insults (29 % ), and necrotizing vasculitis of the skin (29 % ). All patients underwent renal biopsy examined by immunohistochemistry with expression of alpha-smooth muscle actin (alpha SMA) in glomerular and interstitial spaces. Patients were followed from 6 months to 5.5 years (35.4± 23.2 months). None of the patients died. Two of seven children who had ARF progressed to end stage renal disease; one developed chronic renal failure, and four normalized renal function. ARF and central nervous system involvement at presentation were parameters of poor renal outcome. A high score of fibro-cellular glomerular crescents was associated with worse prognosis. Early treatment enables a favorable prognosis of MPO-ANCA-associated MPA in children.