Peco-Antic, Amira (7004525216)Amira (7004525216)Peco-AnticBonaci-Nikolic, Branka (10839652200)Branka (10839652200)Bonaci-NikolicBasta-Jovanovic, Gordana (6603093303)Gordana (6603093303)Basta-JovanovicKostic, Mirjana (56247970900)Mirjana (56247970900)KosticMarkovic-Lipkovski, Jasmina (6603725388)Jasmina (6603725388)Markovic-LipkovskiNikolic, Milos (56910382000)Milos (56910382000)NikolicSpasojevic, Brankica (10839232100)Brankica (10839232100)Spasojevic2025-06-132025-06-132006https://doi.org/10.1007/s00467-005-2063-xhttps://www.scopus.com/inward/record.uri?eid=2-s2.0-29944445112&doi=10.1007%2fs00467-005-2063-x&partnerID=40&md5=572e20af071ea69de4b83567f97320b7https://remedy.med.bg.ac.rs/handle/123456789/11036We 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. © IPNA 2005.Acute renal failureAlpha SMAANCAChildhoodMicroscopic polyangiitis