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Italian Journal of Pediatrics
Published in: Italian Journal of Pediatrics 1/2017

Open Access 01-12-2017 | Review

ANCA-associated vasculitis in childhood: recent advances

Authors: Marta Calatroni, Elena Oliva, Davide Gianfreda, Gina Gregorini, Marco Allinovi, Giuseppe A. Ramirez, Enrica P. Bozzolo, Sara Monti, Claudia Bracaglia, Giulia Marucci, Monica Bodria, Renato A. Sinico, Federico Pieruzzi, Gabriella Moroni, Serena Pastore, Giacomo Emmi, Pasquale Esposito, Mariagrazia Catanoso, Giancarlo Barbano, Alice Bonanni, Augusto Vaglio

Published in: Italian Journal of Pediatrics | Issue 1/2017

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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.
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Metadata
Title
ANCA-associated vasculitis in childhood: recent advances
Authors
Marta Calatroni
Elena Oliva
Davide Gianfreda
Gina Gregorini
Marco Allinovi
Giuseppe A. Ramirez
Enrica P. Bozzolo
Sara Monti
Claudia Bracaglia
Giulia Marucci
Monica Bodria
Renato A. Sinico
Federico Pieruzzi
Gabriella Moroni
Serena Pastore
Giacomo Emmi
Pasquale Esposito
Mariagrazia Catanoso
Giancarlo Barbano
Alice Bonanni
Augusto Vaglio
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Italian Journal of Pediatrics / Issue 1/2017
Electronic ISSN: 1824-7288
DOI
https://doi.org/10.1186/s13052-017-0364-x

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