PT  - JOURNAL ARTICLE
AU  - Seza Ozen
AU  - Angela Pistorio
AU  - Silvia M Iusan
AU  - Aysin Bakkaloglu
AU  - Troels Herlin
AU  - Riva Brik
AU  - Antonella Buoncompagni
AU  - Calin Lazar
AU  - Ilmay Bilge
AU  - Yosef Uziel
AU  - Donato Rigante
AU  - Luca Cantarini
AU  - Maria Odete Hilario
AU  - Clovis A Silva
AU  - Mauricio Alegria
AU  - Ximena Norambuena
AU  - Alexandre Belot
AU  - Yackov Berkun
AU  - Amparo Ibanez Estrella
AU  - Alma Nunzia Olivieri
AU  - Maria Giannina Alpigiani
AU  - Ingrida Rumba
AU  - Flavio Sztajnbok
AU  - Lana Tambic-Bukovac
AU  - Luciana Breda
AU  - Sulaiman Al-Mayouf
AU  - Dimitrina Mihaylova
AU  - Vyacheslav Chasnyk
AU  - Claudia Sengler
AU  - Maria Klein-Gitelman
AU  - Djamal Djeddi
AU  - Laura Nuno
AU  - Chris Pruunsild
AU  - Jurgen Brunner
AU  - Anuela Kondi
AU  - Karaman Pagava
AU  - Silvia Pederzoli
AU  - Alberto Martini
AU  - Nicolino Ruperto
AU  - for the Paediatric Rheumatology International Trials Organisation (PRINTO)
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.