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.
OBJECTIVE
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 <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).


CONCLUSIONS
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.
Ruperto
Nicolino
N
Paediatric Rheumatology International Trials Organisation (PRINTO), IRCCS G Gaslini, Università di Genova, Pediatria II - Reumatologia, EULAR Centre of Excellence in Rheumatology 2008-2013, Largo Gaslini, 5, Genova 16147, Italy. nicolaruperto@ospedale-gaslini.ge.it
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D000293:Adolescent; D001706:Biopsy; D002648:Child; D003697:Delphi Technique; D014890:Granulomatosis with Polyangiitis / Q000145:classification / Q000175:diagnosis*; D006801:Humans; D011695:IgA Vasculitis / Q000145:classification / Q000175:diagnosis*; D007391:International Cooperation; D020407:Internet; D010488:Polyarteritis Nodosa / Q000145:classification / Q000175:diagnosis*; D015203:Reproducibility of Results; D013625:Takayasu Arteritis / Q000145:classification / Q000175:diagnosis*