PT  - JOURNAL ARTICLE
AU  - Ozen, Seza
AU  - Pistorio, Angela
AU  - Iusan, Silvia M
AU  - Bakkaloglu, Aysin
AU  - Herlin, Troels
AU  - Brik, Riva
AU  - Buoncompagni, Antonella
AU  - Lazar, Calin
AU  - Bilge, Ilmay
AU  - Uziel, Yosef
AU  - Rigante, Donato
AU  - Cantarini, Luca
AU  - Hilario, Maria Odete
AU  - Silva, Clovis A
AU  - Alegria, Mauricio
AU  - Norambuena, Ximena
AU  - Belot, Alexandre
AU  - Berkun, Yackov
AU  - Estrella, Amparo Ibanez
AU  - Olivieri, Alma Nunzia
AU  - Alpigiani, Maria Giannina
AU  - Rumba, Ingrida
AU  - Sztajnbok, Flavio
AU  - Tambic-Bukovac, Lana
AU  - Breda, Luciana
AU  - Al-Mayouf, Sulaiman
AU  - Mihaylova, Dimitrina
AU  - Chasnyk, Vyacheslav
AU  - Sengler, Claudia
AU  - Klein-Gitelman, Maria
AU  - Djeddi, Djamal
AU  - Nuno, Laura
AU  - Pruunsild, Chris
AU  - Brunner, Jurgen
AU  - Kondi, Anuela
AU  - Pagava, Karaman
AU  - Pederzoli, Silvia
AU  - Martini, Alberto
AU  - Ruperto, Nicolino
AU  - ,
TI  - EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria
AID  - 10.1136/ard.2009.116657
DP  - 2010 May 01
TA  - Annals of the Rheumatic Diseases
PG  - 798--806
VI  - 69
IP  - 5
4099  - http://ard.bmj.com/content/69/5/798.short
4100  - http://ard.bmj.com/content/69/5/798.full
SO  - Ann Rheum Dis2010 May 01; 69
AB  - Objectives To validate the previously proposed classification criteria for Henoch–Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). Methods Step 1: retrospective/prospective web-data 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 κ-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.