RT Journal Article
SR Electronic
T1 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
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 798
OP 806
DO 10.1136/ard.2009.116657
VO 69
IS 5
A1 Seza Ozen
A1 Angela Pistorio
A1 Silvia M Iusan
A1 Aysin Bakkaloglu
A1 Troels Herlin
A1 Riva Brik
A1 Antonella Buoncompagni
A1 Calin Lazar
A1 Ilmay Bilge
A1 Yosef Uziel
A1 Donato Rigante
A1 Luca Cantarini
A1 Maria Odete Hilario
A1 Clovis A Silva
A1 Mauricio Alegria
A1 Ximena Norambuena
A1 Alexandre Belot
A1 Yackov Berkun
A1 Amparo Ibanez Estrella
A1 Alma Nunzia Olivieri
A1 Maria Giannina Alpigiani
A1 Ingrida Rumba
A1 Flavio Sztajnbok
A1 Lana Tambic-Bukovac
A1 Luciana Breda
A1 Sulaiman Al-Mayouf
A1 Dimitrina Mihaylova
A1 Vyacheslav Chasnyk
A1 Claudia Sengler
A1 Maria Klein-Gitelman
A1 Djamal Djeddi
A1 Laura Nuno
A1 Chris Pruunsild
A1 Jurgen Brunner
A1 Anuela Kondi
A1 Karaman Pagava
A1 Silvia Pederzoli
A1 Alberto Martini
A1 Nicolino Ruperto
A1 for the Paediatric Rheumatology International Trials Organisation (PRINTO)
YR 2010
UL http://ard.bmj.com/content/69/5/798.abstract
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.