TY - JOUR T1 - ANCA-associated vasculitis in childhood: recent advances. JF - Ital J Pediatr Y1 - 2017 A1 - Calatroni, Marta A1 - Oliva, Elena A1 - Gianfreda, Davide A1 - Gregorini, Gina A1 - Allinovi, Marco A1 - Ramirez, Giuseppe A A1 - Bozzolo, Enrica P A1 - Monti, Sara A1 - Bracaglia, Claudia A1 - Marucci, Giulia A1 - Bodria, Monica A1 - Sinico, Renato A A1 - Pieruzzi, Federico A1 - Moroni, Gabriella A1 - Pastore, Serena A1 - Emmi, Giacomo A1 - Esposito, Pasquale A1 - Catanoso, Mariagrazia A1 - Barbano, Giancarlo A1 - Bonanni, Alice A1 - Vaglio, Augusto KW - Age Distribution KW - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis KW - Antibodies, Antineutrophil Cytoplasmic KW - Child KW - Child, Preschool KW - Churg-Strauss Syndrome KW - Female KW - Granulomatosis with Polyangiitis KW - Humans KW - Incidence KW - Male KW - Microscopic Polyangiitis KW - Rare Diseases KW - Risk Assessment KW - Severity of Illness Index KW - Sex Distribution KW - Survival Rate AB -

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.

VL - 43 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/28476172?dopt=Abstract ER -