engleski

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.

Objectives were 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). 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. 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. 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.

criteria; children; Henoch-Schönlein purpura; childhood polyarteritis nodosa; Wegener granulomatosis; Takayasu arteritis; EULAR; PRINTO; PRES

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