Childhood microscopic polyangiitis associated with MPO-ANCA.
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.
Peco-Antic
Amira
A
Department of Nephrology, University Children's Hospital, Tirsova 10, 11000 Belgrade, Serbia. amirapecoantic@yahoo.com
Bonaci-Nikolic
Branka
B
Basta-Jovanovic
Gordana
G
Kostic
Mirjana
M
Markovic-Lipkovski
Jasmina
J
Nikolic
Milos
M
Spasojevic
Brankica
B
D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D002648:Child; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000276:immunology*; D006801:Humans; D007377:Interleukin-3 / Q000276:immunology*; D008297:Male; D011993:Recombinant Fusion Proteins / Q000276:immunology*; D011994:Recombinant Proteins; D012189:Retrospective Studies; D014657:Vasculitis / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy
D019268:Antibodies, Antineutrophil Cytoplasmic; D007377:Interleukin-3; D011993:Recombinant Fusion Proteins; D011994:Recombinant Proteins; C404145:myelopoietin; D016179:Granulocyte Colony-Stimulating Factor