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Review
. 2017 May 5;43(1):46.
doi: 10.1186/s13052-017-0364-x.

ANCA-associated vasculitis in childhood: recent advances

Marta Calatroni  1 , Elena Oliva  2 , Davide Gianfreda  2 , Gina Gregorini  3 , Marco Allinovi  4 , Giuseppe A Ramirez  5 , Enrica P Bozzolo  5 , Sara Monti  6 , Claudia Bracaglia  7 , Giulia Marucci  7 , Monica Bodria  8   9 , Renato A Sinico  10 , Federico Pieruzzi  10 , Gabriella Moroni  11 , Serena Pastore  12 , Giacomo Emmi  13 , Pasquale Esposito  1 , Mariagrazia Catanoso  14 , Giancarlo Barbano  8 , Alice Bonanni  8 , Augusto Vaglio  15
Affiliations
Review

ANCA-associated vasculitis in childhood: recent advances

Marta Calatroni et al. Ital J Pediatr. .

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome. Herein, we review the main clinical and therapeutic aspects of childhood-onset ANCA-associated vasculitis, and provide preliminary data on demographic characteristics and organ manifestations of an Italian multicentre cohort.

Keywords: ANCA; Autoimmunity; Childhood; Glomerulonephritis; Renal failure; Vasculitis.

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