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*, 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 OlivieriMaria 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

*Corresponding author for this work

Research output: Contribution to journalArticle

598 Scopus citations

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 webdata 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.

Original languageEnglish (US)
Pages (from-to)798-806
Number of pages9
JournalAnnals of the Rheumatic Diseases
Volume69
Issue number5
DOIs
StatePublished - May 2010

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)

Fingerprint Dive into the research topics of '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'. Together they form a unique fingerprint.

  • Cite this

    Ozen, S., Pistorio, A., Iusan, S. M., Bakkaloglu, A., Herlin, T., Brik, R., Buoncompagni, A., Lazar, C., Bilge, I., Uziel, Y., Rigante, D., Cantarini, L., Hilario, M. O., Silva, C. A., Alegria, M., Norambuena, X., Belot, A., Berkun, Y., Estrella, A. I., ... Ruperto, N. (2010). 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. Annals of the Rheumatic Diseases, 69(5), 798-806. https://doi.org/10.1136/ard.2009.116657