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RESEARCH PRODUCT

EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

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EULAR/PRINTO/PRES Objectives To validate the previously proposed classification criteria for Henoch-Schonlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA).Methods Step 1: retrospective/prospective webdata collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis <= 18 years. Step 2: blinded classification by consensus panel of a representative sample of 280 cases. Step 3: statistical (sensitivity, specificity, area under the curve and.-agreement) and nominal group technique consensus evaluations.Results 827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classified as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classification of c-PAN required a systemic inflammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of five criteria: (1) skin involvement; (2) myalgia/muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classification of c-WG required three of six criteria: (1) histopathological evidence of granulomatous inflammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classification of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of five criteria: (1) pulse deficit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) elevated acute phase reactant.Conclusion European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society propose validated classification criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specificity. Hacettepe Univ, Childrens Hosp, Dept Pediat Nephrol & Rheumatol, TR-06100 Ankara, Turkey IRCCS G Gaslini, Serv Epidemiol & Biostat, Genoa, Italy PRINTO, IRCCS G Gaslini, Genoa, Italy Aarhus Univ Hosp, Dept Pediat, Skejby Sygehus, DK-8000 Aarhus N, Denmark Rambam Med Ctr, Dept Pediat B, Haifa, Israel Clin Pediat 1, Cluj Napoca, Romania Istanbul Univ, Dept Paediat Nephrol, Istanbul, Turkey Meir Med Ctr, Dept Paediat, Kefar Sava, Israel Univ Cattolica Sacro Cuore, Dept Paediat Sci, Rome, Italy Med Clin & Sci Immunol Ist, Policlin Le Scotte, Siena, Italy Universidade Federal de São Paulo, Dept Pediat, São Paulo, Brazil Univ São Paulo, Inst Crianca, São Paulo, Brazil Univ São Paulo, Fac Med, Div Rheumatol, São Paulo, Brazil Hosp Ninos Benjamin Bloom, San Salvador, El Salvador Hosp Dr Exequiel Gonzalez Cortes, Serv Pediat, Unidad Inmunoreumatol Pediat, Santiago, Chile Hop Femme Mere Enfant, Lyon, France Safra Childrens Hosp, Sheba Med Ctr, Tel Hashomer, Israel Inst Salud Nino, Serv Reumatol, Lima, Peru Univ Naples 2, Dipartimento Pedit F Fede, Naples, Italy IRCCS Ist G Gaslini, Clin Pediat 1, Genoa, Italy Latvian State Univ, LV-1063 Riga, Latvia Hosp Univ Pedro Ernesto, Rio de Janeiro, Brazil Univ Zagreb, Dept Paediat, Div Rheumatol, Ctr Hosp,Univ Sch Med, Zagreb 41000, Croatia Univ G dAnnunzio, Osped Policlin, Dipartimento Pediat, Chieti, Italy King Faisal Specialist Hosp & Res Ctr, Dept Paediat, Riyadh 11211, Saudi Arabia Univ Children Hosp, Dept Paediat Rheumatol, Sofia, Bulgaria Hosp Paediat Dept, St Petersburg State Pediat Med Acad, St Petersburg, Russia Charite, D-13353 Berlin, Germany Childrens Mem Hosp, Div Rheumatol, Chicago, IL 60614 USA Dept Pediat, Amiens, France Hosp Gen Univ La Paz, Madrid, Spain Tartu Univ Hosp, Dept Gen Pediat, Childrens Clin, Tartu, Estonia Med Univ, Dept Paediat, Innsbruck, Austria Tblisi State Med Univ, Tbilisi Hosp 1, Dept Paediat & Adolescent Med, Tbilisi, Rep of Georgia Tblisi State Med Univ, Georgian Sci Pract Rheumatol Ctr, Tbilisi, Rep of Georgia Univ Genoa, Dipartimento Pediat, Genoa, Italy Universidade Federal de São Paulo, Dept Pediat, São Paulo, Brazil Web of Science