Journal Article

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

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

2010

Overview

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.

• Publisher: BMJ Publishing Group Ltd and European League Against Rheumatism,Elsevier Limited
• Subject: Adolescent / Age / Agreements / Biopsy / Child / Classification / Coronary vessels / Data collection / Disease / Epidemiologic Methods / Granulomatosis with Polyangiitis - classification / Granulomatosis with Polyangiitis - diagnosis / Humans / Hypertension / IgA Vasculitis - classification / IgA Vasculitis - diagnosis / International Cooperation / Laboratories / Physicians / Polyarteritis Nodosa - classification / Polyarteritis Nodosa - diagnosis / Rheumatology / Takayasu Arteritis - classification / Takayasu Arteritis - diagnosis / Terminology as Topic