Pediatric Nephrology

, Volume 21, Issue 1, pp 46–53

Childhood microscopic polyangiitis associated with MPO-ANCA

Authors

    • Department of NephrologyUniversity Children’s Hospital
  • Branka Bonaci-Nikolic
    • Institute of Allergy and Clinical ImmunologyClinical Centre of Serbia
  • Gordana Basta-Jovanovic
    • Institute of Allergy and Clinical ImmunologyClinical Centre of Serbia
  • Mirjana Kostic
    • Department of NephrologyUniversity Children’s Hospital
  • Jasmina Markovic-Lipkovski
    • Institute of PathologySchool of Medicine, University of Belgrade
  • Milos Nikolic
    • Clinical Centre of SerbiaInstitute of Dermatology
  • Brankica Spasojevic
    • Department of NephrologyUniversity Children’s Hospital
Original Article

DOI: 10.1007/s00467-005-2063-x

Cite this article as:
Peco-Antic, A., Bonaci-Nikolic, B., Basta-Jovanovic, G. et al. Pediatr Nephrol (2006) 21: 46. doi:10.1007/s00467-005-2063-x

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.

Keywords

Acute renal failureAlpha SMAANCAChildhoodMicroscopic polyangiitis

Copyright information

© IPNA 2005