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.
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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, Kone-Paut Isabella, 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, Paediatric Rheumatology International Trials Organisation (PRINTO)
Аннотация
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 <or=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, kappa-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 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.