Anti-neutrophil cytoplasmic auto-antibodies-associated vasculitis with pulmonary and renal involvement.
We present a 13-year-old boy with a rapidly progressive glomerulonephritis and pulmonary haemorrhage with perinuclear anti-neutrophil cytoplasmic auto-antibodies (pANCA) corresponding to anti-myeloperoxidase antibodies. The diagnosis of microscopic polyarteritis was made on the basis of the clinical features, the positivity of pANCA, and the histological finding of a pauci-immune crescentic glomerulonephritis. He responded excellently to corticosteroids and cyclophosphamide therapy and complete clinical remission persists 1 year after withdrawal of treatment. We emphasize the usefulness of ANCA antibody assays to establish a prompt diagnosis and adequate treatment in systemic vasculitis in children.
Pintos-Morell
G
G
Department of Paediatrics, University Hospital Germans Trias i Pujol, Badalona, Spain.
Roca-Comas
A
A
Naranjo
M A
MA
Tural
C
C
Abad
E
E
Javier
G
G
Prats
J
J
D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D003593:Cytoplasm / Q000276:immunology*; D005921:Glomerulonephritis / Q000150:complications* / Q000276:immunology; D006470:Hemorrhage / Q000150:complications* / Q000276:immunology; D006801:Humans; D008171:Lung Diseases / Q000150:complications* / Q000276:immunology; D008297:Male; D009504:Neutrophils / Q000276:immunology*; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000276:immunology; D011650:Pulmonary Alveoli
D001323:Autoantibodies