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Childhood microscopic polyangiitis associated with MPO-ANCA

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Abstract

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.

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Authors and Affiliations

  1. Department of Nephrology, University Children’s Hospital, Tirsova 10, 11000, Belgrade, Serbia

    Amira Peco-Antic, Mirjana Kostic & Brankica Spasojevic

  2. Institute of Allergy and Clinical Immunology, Clinical Centre of Serbia, Belgrade, Serbia

    Branka Bonaci-Nikolic & Gordana Basta-Jovanovic

  3. Institute of Pathology, School of Medicine, University of Belgrade, Belgrade, Serbia

    Jasmina Markovic-Lipkovski

  4. Clinical Centre of Serbia, Institute of Dermatology, Belgrade, Serbia

    Milos Nikolic

Authors
  1. Amira Peco-Antic
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  2. Branka Bonaci-Nikolic
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  4. Mirjana Kostic
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  6. Milos Nikolic
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Correspondence to Amira Peco-Antic.

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Peco-Antic, A., Bonaci-Nikolic, B., Basta-Jovanovic, G. et al. Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol 21, 46–53 (2006). https://doi.org/10.1007/s00467-005-2063-x

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  • DOI: https://doi.org/10.1007/s00467-005-2063-x

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