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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
  1. Seza Ozen1,
  2. Angela Pistorio2,
  3. Silvia M Iusan3,
  4. Aysin Bakkaloglu1,
  5. Troels Herlin4,
  6. Riva Brik5,
  7. Antonella Buoncompagni3,
  8. Calin Lazar6,
  9. Ilmay Bilge7,
  10. Yosef Uziel8,
  11. Donato Rigante9,
  12. Luca Cantarini10,
  13. Maria Odete Hilario11,
  14. Clovis A Silva12,
  15. Mauricio Alegria13,
  16. Ximena Norambuena14,
  17. Alexandre Belot15,
  18. Yackov Berkun16,
  19. Amparo Ibanez Estrella17,
  20. Alma Nunzia Olivieri18,
  21. Maria Giannina Alpigiani19,
  22. Ingrida Rumba20,
  23. Flavio Sztajnbok21,
  24. Lana Tambic-Bukovac22,
  25. Luciana Breda23,
  26. Sulaiman Al-Mayouf24,
  27. Dimitrina Mihaylova25,
  28. Vyacheslav Chasnyk26,
  29. Claudia Sengler27,
  30. Maria Klein-Gitelman28,
  31. Djamal Djeddi29,
  32. Laura Nuno30,
  33. Chris Pruunsild31,
  34. Jurgen Brunner32,
  35. Anuela Kondi3,
  36. Karaman Pagava33,
  37. Silvia Pederzoli3,
  38. Alberto Martini3,34,
  39. Nicolino Ruperto3,
  40. for the Paediatric Rheumatology International Trials Organisation (PRINTO)
  1. 1Hacettepe University Children's Hospital, Ankara, Turkey
  2. 2IRCCS G Gaslini, Servizio di Epidemiologia e Biostatistica, Genova, Italy
  3. 3IRCCS G Gaslini, Pediatria II, Reumatologia, PRINTO, Genova, Italy
  4. 4Skejby Sygehus, Department of Pediatrics, Aarhus University Hospital, Aarhus N, Denmark
  5. 5Department of Pediatrics B, Rambam Medical Centre, Haifa, Israel
  6. 6Clinica Pediatrie I, Cluj-Napoca, Romania
  7. 7Department of Paediatric Nephrology, Istanbul University, Istanbul, Turkey
  8. 8Department of Paediatrics, Meir Medical Centre, Kfar Saba, Israel
  9. 9Department of Paediatric Sciences, Università Cattolica del Sacro Cuore, Roma, Italy
  10. 10Policlinico Le Scotte, Medicina Clinica e Scienze Immunologische Ist. Reumatologia, Siena, Italy
  11. 11Departamento De Pediatria, Universitade Federal de Sao Paolo, Sao Paulo, Brazil
  12. 12Instituto da Criança and Division of Rheumatology – Faculdade de Medicina da Universidade de São Paulo, Sao Paulo, Brazil
  13. 13Hospital de Niños Benjamin Bloom, Rheumatology, San Salvador, El Salvador
  14. 14Hospital Dr Exequiel Gonzalez Cortes, Servicio de Pediatria – Unidad de Inmuno- Reumatologia Pediatrica, Santiago, Chile
  15. 15Hopital Femme-Mère-Enfant, Rhumatologie Pédiatrique, Bron (Lyon), France
  16. 16Safra Childrens Hospital, Sheba Medical Centre, Tel Hashomer, Israel
  17. 17Instituto de Salud del Nino, Servicio de Reumatologia, Breña, Lima, Peru
  18. 18Dipartimento di Pediatria F Fede, Seconda Universita' degli Studi di Napoli, Napoli, Italy
  19. 19IRCCS Istituto G Gaslini, Clinica Pediatrica I, Genova, Italy
  20. 20Pediatric Rheumatology, University of Latvia, Riga, Latvia
  21. 21Hospital Universitario Pedro Ernesto, Nucleo de Estudos da saude do adolescente, Rio de Janeiro, Brazil
  22. 22Department of Paediatrics, Division of Rheumatology, Zagreb University Hospital Centre, University School of Medicine, Zagreb, Croatia
  23. 23Dipartimento di Pediatria, Ospedale Policlinico – Universita' degli studi di Chieti, Chieti, Italy
  24. 24Department of Paediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
  25. 25Department of Paediatric Rheumatology, University Children Hospital, Sofia, Bulgaria
  26. 26Saint-Petersburg State Pediatric Medical Academy, Hospital Paediatric Department, Saint-Petersburg, Russia
  27. 27Charité-Universitätmedizin Berlin, Ped Rheumatology, Berlin, Germany
  28. 28Division of Rheumatology, Children's Memorial Hospital, Chicago, Illinois, USA
  29. 29Département de pédiatrie, Place V Pauchet, Amiens, France
  30. 30Hospital General Universitario La Paz, Rheumatology, Madrid, Spain
  31. 31Department of General Pediatrics, Tartu University Hospital, Children's Clinic, Tartu, Estonia
  32. 32Department of Paediatrics, Medical University, Innsbruck, Austria
  33. 33Department of Paediatrics and Adolescent Medicine, Tbilisi Hospital #1 and Georgian Scientific-Practical Rheumatology Centre, Tblisi State Medical University, Tbilisi, Georgia
  34. 34Dipartimento di Pediatria, Università degli Studi, Genova, Italy
  1. Correspondence to Professor Seza Ozen, Hacettepe University Children's Hospital, Department of Pediatric Nephrology and Rheumatology, Sihhiye, Ankara 06100, Turkey; sezaozen{at}hacettepe.edu.tr

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.

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Footnotes

  • Competing interests None.

  • Funding EULAR/PRINTO/PRES.

  • Ethics approval This study was conducted with the approval of all participating centres if required by the national laws of the specific country.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • This is the final product of more than 5 years of work supported by EULAR/PRINTO and PRES in order to propose new classification criteria for childhood vasculitis.

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