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. Issue 5 (22nd April 2010)

• Record Type: Journal Article
• Authors: Ozen, Seza; Pistorio, Angela; Iusan, Silvia M; Bakkaloglu, Aysin; Herlin, Troels; Brik, Riva; Buoncompagni, Antonella; Lazar, Calin; Bilge, Ilmay; Uziel, Yosef; Rigante, Donato; Cantarini, Luca; Hilario, Maria Odete; Silva, Clovis A; Alegria, Mauricio; Norambuena, Ximena; Belot, Alexandre; Berkun, Yackov; Estrella, Amparo Ibanez; Olivieri, Alma Nunzia; Alpigiani, Maria Giannina; Rumba, Ingrida; Sztajnbok, Flavio; Tambic-Bukovac, Lana; Breda, Luciana; Al-Mayouf, Sulaiman; Mihaylova, Dimitrina; Chasnyk, Vyacheslav; Sengler, Claudia; Klein-Gitelman, Maria; Djeddi, Djamal; Nuno, Laura; Pruunsild, Chris; Brunner, Jurgen; Kondi, Anuela; Pagava, Karaman; Pederzoli, Silvia; Martini, Alberto; Ruperto, Nicolino;
• Is Part Of: Annals of the rheumatic diseases. Volume 69:Issue 5(2010)
• Page Start: 798
• Page End: 806
• Publication Date: 2010-04-22
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/ard.2009.116657
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : 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.