@article {10533, title = {ANCA-associated vasculitis in childhood: recent advances.}, journal = {Ital J Pediatr}, volume = {43}, year = {2017}, month = {2017 May 05}, pages = {46}, abstract = {

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener{\textquoteright}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 = {Age Distribution, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Antineutrophil Cytoplasmic, Child, Child, Preschool, Churg-Strauss Syndrome, Female, Granulomatosis with Polyangiitis, Humans, Incidence, Male, Microscopic Polyangiitis, Rare Diseases, Risk Assessment, Severity of Illness Index, Sex Distribution, Survival Rate}, issn = {1824-7288}, doi = {10.1186/s13052-017-0364-x}, author = {Calatroni, Marta and Oliva, Elena and Gianfreda, Davide and Gregorini, Gina and Allinovi, Marco and Ramirez, Giuseppe A and Bozzolo, Enrica P and Monti, Sara and Bracaglia, Claudia and Marucci, Giulia and Bodria, Monica and Sinico, Renato A and Pieruzzi, Federico and Moroni, Gabriella and Pastore, Serena and Emmi, Giacomo and Esposito, Pasquale and Catanoso, Mariagrazia and Barbano, Giancarlo and Bonanni, Alice and Vaglio, Augusto} } @article {1695, title = {EULAR/PRINTO/PRES criteria for Henoch-Sch{\"o}nlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation.}, journal = {Ann Rheum Dis}, volume = {69}, year = {2010}, month = {2010 May}, pages = {790-7}, abstract = {

OBJECTIVES: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Sch{\"o}nlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

METHODS: The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis

RESULTS: A total of 1183/1398 (85\%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a kappa-agreement of 0.96 for HSP (95\% CI 0.84 to 1), 0.88 for c-WG (95\% CI 0.76 to 0.99), 0.84 for c-TA (95\% CI 0.73 to 0.96) and 0.73 for c-PAN (95\% CI 0.62 to 0.84), with an overall kappa of 0.79 (95\% CI 0.73 to 0.84).

CONCLUSION: EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

}, keywords = {Adolescent, Biopsy, Child, Delphi Technique, Granulomatosis with Polyangiitis, Humans, International Cooperation, Internet, Polyarteritis Nodosa, Purpura, Schoenlein-Henoch, Reproducibility of Results, Takayasu Arteritis}, issn = {1468-2060}, doi = {10.1136/ard.2009.116624}, author = {Ruperto, Nicolino and Ozen, Seza and Pistorio, Angela and Dolezalova, Pavla and Brogan, Paul and Cabral, David A and Cuttica, Ruben and Khubchandani, Raju and Lovell, Daniel J and O{\textquoteright}Neil, Kathleen M and Quartier, Pierre and Ravelli, Angelo and Iusan, Silvia M and Filocamo, Giovanni and Magalh{\~a}es, Claudia Saad and Unsal, Erbil and Oliveira, Sheila and Bracaglia, Claudia and Bagga, Arvind and Stanevicha, Valda and Manzoni, Silvia Magni and Pratsidou, Polyxeni and Lepore, Loredana and Espada, Graciela and Kone-Paut, Isabella and Paut, Isabelle Kone and Zulian, Francesco and Barone, Patrizia and Bircan, Zelal and Maldonado, Maria del Rocio and Russo, Ricardo and Vilca, Iris and Tullus, Kjell and Cimaz, Rolando and Horneff, Gerd and Anton, Jordi and Garay, Stella and Nielsen, Susan and Barbano, Giancarlo and Martini, Alberto} }