TY - JOUR
T1 - ANCA-associated vasculitis in childhood
T2 - Recent advances
AU - Calatroni, Marta
AU - Oliva, Elena
AU - Gianfreda, Davide
AU - Gregorini, Gina
AU - Allinovi, Marco
AU - Ramirez, Giuseppe A.
AU - Bozzolo, Enrica P.
AU - Monti, Sara
AU - Bracaglia, Claudia
AU - Marucci, Giulia
AU - Bodria, Monica
AU - Sinico, Renato A.
AU - Pieruzzi, Federico
AU - Moroni, Gabriella
AU - Pastore, Serena
AU - Emmi, Giacomo
AU - Esposito, Pasquale
AU - Catanoso, Mariagrazia
AU - Barbano, Giancarlo
AU - Bonanni, Alice
AU - Vaglio, Augusto
PY - 2017/5/5
Y1 - 2017/5/5
N2 - 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.
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.
KW - ANCA
KW - Autoimmunity
KW - Childhood
KW - Glomerulonephritis
KW - Renal failure
KW - Vasculitis
UR - http://www.scopus.com/inward/record.url?scp=85019016037&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85019016037&partnerID=8YFLogxK
U2 - 10.1186/s13052-017-0364-x
DO - 10.1186/s13052-017-0364-x
M3 - Review article
AN - SCOPUS:85019016037
VL - 43
JO - Italian Journal of Pediatrics
JF - Italian Journal of Pediatrics
SN - 1720-8424
IS - 1
M1 - 46
ER -