%0 Journal Article %J Ann Rheum Dis %D 2010 %T EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation. %A Ruperto, Nicolino %A Ozen, Seza %A Pistorio, Angela %A Dolezalova, Pavla %A Brogan, Paul %A Cabral, David A %A Cuttica, Ruben %A Khubchandani, Raju %A Lovell, Daniel J %A O'Neil, Kathleen M %A Quartier, Pierre %A Ravelli, Angelo %A Iusan, Silvia M %A Filocamo, Giovanni %A Magalhães, Claudia Saad %A Unsal, Erbil %A Oliveira, Sheila %A Bracaglia, Claudia %A Bagga, Arvind %A Stanevicha, Valda %A Manzoni, Silvia Magni %A Pratsidou, Polyxeni %A Lepore, Loredana %A Espada, Graciela %A Kone-Paut, Isabella %A Paut, Isabelle Kone %A Zulian, Francesco %A Barone, Patrizia %A Bircan, Zelal %A Maldonado, Maria del Rocio %A Russo, Ricardo %A Vilca, Iris %A Tullus, Kjell %A Cimaz, Rolando %A Horneff, Gerd %A Anton, Jordi %A Garay, Stella %A Nielsen, Susan %A Barbano, Giancarlo %A Martini, Alberto %K Adolescent %K Biopsy %K Child %K Delphi Technique %K Granulomatosis with Polyangiitis %K Humans %K International Cooperation %K Internet %K Polyarteritis Nodosa %K Purpura, Schoenlein-Henoch %K Reproducibility of Results %K Takayasu Arteritis %X

OBJECTIVES: To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein 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.

%B Ann Rheum Dis %V 69 %P 790-7 %8 2010 May %G eng %N 5 %1 http://www.ncbi.nlm.nih.gov/pubmed/20388738?dopt=Abstract %R 10.1136/ard.2009.116624