Original Article

Pediatric Nephrology

, Volume 21, Issue 1, pp 46-53

First online:

Childhood microscopic polyangiitis associated with MPO-ANCA

  • Amira Peco-AnticAffiliated withDepartment of Nephrology, University Children’s Hospital Email author 
  • , Branka Bonaci-NikolicAffiliated withInstitute of Allergy and Clinical Immunology, Clinical Centre of Serbia
  • , Gordana Basta-JovanovicAffiliated withInstitute of Allergy and Clinical Immunology, Clinical Centre of Serbia
  • , Mirjana KosticAffiliated withDepartment of Nephrology, University Children’s Hospital
  • , Jasmina Markovic-LipkovskiAffiliated withInstitute of Pathology, School of Medicine, University of Belgrade
  • , Milos NikolicAffiliated withClinical Centre of Serbia, Institute of Dermatology
  • , Brankica SpasojevicAffiliated withDepartment of Nephrology, University Children’s Hospital

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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.


Acute renal failure Alpha SMA ANCA Childhood Microscopic polyangiitis