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.
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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, Paediatric Rheumatology International Trials Organisation (PRINTO)
Аннотация
METHODS: Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis <or=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 kappa-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.