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. 2010 May;69(5):798-806.
doi: 10.1136/ard.2009.116657.

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

Seza Ozen  1 , Angela Pistorio, Silvia M Iusan, Aysin Bakkaloglu, Troels Herlin, Riva Brik, Antonella Buoncompagni, Calin Lazar, Ilmay Bilge, Yosef Uziel, Donato Rigante, Luca Cantarini, Maria Odete Hilario, Clovis A Silva, Mauricio Alegria, Ximena Norambuena, Alexandre Belot, Yackov Berkun, Amparo Ibanez Estrella, Alma Nunzia Olivieri, Maria Giannina Alpigiani, Ingrida Rumba, Flavio Sztajnbok, Lana Tambic-Bukovac, Luciana Breda, Sulaiman Al-Mayouf, Dimitrina Mihaylova, Vyacheslav Chasnyk, Claudia Sengler, Maria Klein-Gitelman, Djamal Djeddi, Laura Nuno, Chris Pruunsild, Jurgen Brunner, Anuela Kondi, Karaman Pagava, Silvia Pederzoli, Alberto Martini, Nicolino Ruperto; Paediatric Rheumatology International Trials Organisation (PRINTO)
Affiliations

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

Seza Ozen et al. Ann Rheum Dis. 2010 May.

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 <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.

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