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. Issue 5 (13th April 2010)

• Record Type: Journal Article
• Authors: Ruperto, Nicolino; Ozen, Seza; Pistorio, Angela; Dolezalova, Pavla; Brogan, Paul; Cabral, David A; Cuttica, Ruben; Khubchandani, Raju; Lovell, Daniel J; O'Neil, Kathleen M; Quartier, Pierre; Ravelli, Angelo; Iusan, Silvia M; Filocamo, Giovanni; Magalhães, Claudia Saad; Unsal, Erbil; Oliveira, Sheila; Bracaglia, Claudia; Bagga, Arvind; Stanevicha, Valda; Manzoni, Silvia Magni; Pratsidou, Polyxeni; Lepore, Loredana; Espada, Graciela; Paut, Isabelle Kone; Zulian, Francesco; Barone, Patrizia; Bircan, Zelal; Maldonado, Maria del Rocio; Russo, Ricardo; Vilca, Iris; Tullus, Kjell; Cimaz, Rolando; Horneff, Gerd; Anton, Jordi; Garay, Stella; Nielsen, Susan; Barbano, Giancarlo; Martini, Alberto;
• Is Part Of: Annals of the rheumatic diseases. Volume 69:Issue 5(2010)
• Page Start: 790
• Page End: 797
• Publication Date: 2010-04-13
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/ard.2009.116624
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : 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 ≤18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, κ-agreement) using as 'gold standard' the final consensus classification or original treating physician 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 κ-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 κ 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.