ANCA-associated vasculitis in childhood: recent advances

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

来源：Italian Journal of Pediatrics, 2017, 43(1): 46.

DOI：10.1186/s13052-017-0364-x

• ANCA
• Vasculitis
• Glomerulonephritis
• Childhood
• Renal failure
• Autoimmunity

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. ANCAassociated 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.

• 出版日期2017-5-5