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 Ozen, Seza
A1 Pistorio, Angela
A1 Iusan, Silvia M
A1 Bakkaloglu, Aysin
A1 Herlin, Troels
A1 Brik, Riva
A1 Buoncompagni, Antonella
A1 Lazar, Calin
A1 Bilge, Ilmay
A1 Uziel, Yosef
A1 Rigante, Donato
A1 Cantarini, Luca
A1 Hilario, Maria Odete
A1 Silva, Clovis A
A1 Alegria, Mauricio
A1 Norambuena, Ximena
A1 Belot, Alexandre
A1 Berkun, Yackov
A1 Estrella, Amparo Ibanez
A1 Olivieri, Alma Nunzia
A1 Alpigiani, Maria Giannina
A1 Rumba, Ingrida
A1 Sztajnbok, Flavio
A1 Tambic-Bukovac, Lana
A1 Breda, Luciana
A1 Al-Mayouf, Sulaiman
A1 Mihaylova, Dimitrina
A1 Chasnyk, Vyacheslav
A1 Sengler, Claudia
A1 Klein-Gitelman, Maria
A1 Djeddi, Djamal
A1 Nuno, Laura
A1 Pruunsild, Chris
A1 Brunner, Jurgen
A1 Kondi, Anuela
A1 Pagava, Karaman
A1 Pederzoli, Silvia
A1 Martini, Alberto
A1 Ruperto, Nicolino
A1 ,
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.