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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 I: Overall methodology and clinical characterisation
  1. Nicolino Ruperto1,
  2. Seza Ozen2,
  3. Angela Pistorio3,
  4. Pavla Dolezalova4,
  5. Paul Brogan5,
  6. David A Cabral6,
  7. Ruben Cuttica7,
  8. Raju Khubchandani8,
  9. Daniel J Lovell9,
  10. Kathleen M O'Neil10,
  11. Pierre Quartier11,
  12. Angelo Ravelli1,12,
  13. Silvia M Iusan1,
  14. Giovanni Filocamo1,
  15. Claudia Saad Magalhães13,
  16. Erbil Unsal14,
  17. Sheila Oliveira15,
  18. Claudia Bracaglia16,
  19. Arvind Bagga17,
  20. Valda Stanevicha18,
  21. Silvia Magni Manzoni19,
  22. Polyxeni Pratsidou20,
  23. Loredana Lepore21,
  24. Graciela Espada22,
  25. Isabelle Kone Paut23,
  26. Francesco Zulian24,
  27. Patrizia Barone25,
  28. Zelal Bircan26,
  29. Maria del Rocio Maldonado27,
  30. Ricardo Russo28,
  31. Iris Vilca1,28,
  32. Kjell Tullus29,
  33. Rolando Cimaz30,
  34. Gerd Horneff31,
  35. Jordi Anton32,
  36. Stella Garay33,
  37. Susan Nielsen34,
  38. Giancarlo Barbano35,
  39. Alberto Martini1,12,
  40. for the Paediatric Rheumatology International Trials Organisation (PRINTO)
  1. 1IRCCS G Gaslini, Pediatria II, Reumatologia, PRINTO, Genova, Italy
  2. 2Hacettepe University Children's Hospital, Ankara, Turkey
  3. 3IRCCS G Gaslini, Servizio di Epidemiologia e Biostatistica, Genova, Italy
  4. 4Department of Paediatrics and Adolescent Medicine, 1st Faculty of Medicine and General University Hospital, Praha, Czech Republic
  5. 5Great Ormond St Hospital, Rheumatology, London, UK
  6. 6British Columbia Children's Hospital, Vancouver, Canada
  7. 7Rheumatology Section, Hospital General de Ninos Pedro de Elizalde, Buenos Aires, Argentina
  8. 8Department of Paediatrics, Jaslok Hospital and Research Centre, Mumbai, India
  9. 9Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, USA
  10. 10Children's Hospital at OUMC, University of Oklahoma, Health Sciences Center, Oklahoma City, USA
  11. 11Universite Paris-Descartes and Centre de reference national pour les Arthrites Juveniles. Unite d'Immunologie, Hematologie et Rhumatologie Pediatriques, Hopital Necker-Enfants Malades, Paris, France
  12. 12Dipartimento di Pediatria, Università degli Studi, Genova, Italy
  13. 13Departamento de Pediatria – UNESP, Hospital das Clínicas – Faculdade de Medicina de Botucatu, UNESP, Botucatu, Brazil
  14. 14Dokuz EyluL University Medical Faculty, Division of Pediatric Rheumatology–Immunology, Balcova, Izmir, Turkey
  15. 15Universidade Federal do Rio de Janeiro, Instituto de Puericultura e Pediatria Martagao Gesteira, Rio de Janeiro, Brazil
  16. 16Pediatria II/Reumatologia – IRCCS, Ospedale Pediatrico Bambino Gesù, Roma, Italia
  17. 17All India Institute of Medical Sciences
  18. 18Riga Stradins University, Pediatric, Riga, Latvia
  19. 19IRCCS Policlinico S Matteo, Clinica Pediatrica, Pavia, Italy
  20. 20Department of Pediatrics Ippokration Hospital, Aristotle University of Thessaloniki – Ippokration General Hospital, Thessaloniki, Greece
  21. 21Universita' degli Studi di Trieste, Scienze della Riproduzione e dello Sviluppo, Trieste, Italy
  22. 22Hospital de Ninos Ricardo Gutierrez, Rheumatology Section HNRG Buenos Aires University, Buenos Aires, Argentina
  23. 23Centre de référence national des maladies auto-inflammatoires, Service de pédiatrie générale, rhumatologie pédiatrique, Le Kremlin Bicetre (Paris), France
  24. 24Department of Pediatrics, University of Padua, Padua, Italy
  25. 25Azienda Policlinico Universita' di Catania, Clinica Pediatrica, Catania, Italy
  26. 26Kocaeli University Hospital, Pediatric Nephrology, Kocaeli, Turkey
  27. 27Hospital Infantil de Mexico, Medicina Interna y Reumatologia, Mexico City, Mexico
  28. 28Hospital de Pediatria Juan P Garrahan, Servicio de Inmunología/Reumatología, Buenos Aires, Argentina
  29. 29Great Ormond Street Hospital, Renal Unit, London, UK
  30. 30Dipartimento di Pediatria, Ospedale A Meyer, Firenze, Italy
  31. 31Zentrum für Allgemeine Paediatrie und Neonatologie, Sankt Augustin, Germany
  32. 32Hospital Sant Joan de Déu, Pediatric Rheumatology Unit, Universitat de Barcelona, Esplugues, Barcelona, Spain
  33. 33Hospital Sor Maria Ludovica, Unidad de Reumatologia, La Plata, Argentina
  34. 34Juliane Marie Centret, Rigshospitalet, Juliane Marie Centret, Rigshospitalet, København, Denmark
  35. 35IRCCS Istituto G Gaslini, Nefrologia, Genova, Italy
  1. Correspondence to Dr Nicolino Ruperto, Paediatric Rheumatology International Trials Organisation (PRINTO), IRCCS G Gaslini, Università di Genova, Pediatria II – Reumatologia, EULAR Centre of Excellence in Rheumatology 2008-2013, Largo Gaslini, 5, Genova 16147, Italy; nicolaruperto{at}ospedale-gaslini.ge.it

Abstract

Objectives To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch–Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

Methods The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis ≤18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, κ-agreement) using as ‘gold standard’ the final consensus classification or original treating physician diagnosis.

Results A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a κ-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall κ of 0.79 (95% CI 0.73 to 0.84).

Conclusion EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

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Footnotes

  • Funding EULAR/PRINTO/PRES.

  • Competing interests None.

  • 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.

  • Principal investigator Seza Ozen, MD, Ankara, Turkey. Project coordinator: Nicolino Ruperto, MD, MPH, Genoa, Italy. Vienna (Austria) Consensus Conference10 Consensus moderator: Nicolino Ruperto, MD, MPH, Genoa, Italy. Consensus participants: Seza Ozen, MD, Ankara, Turkey, Principal Investigator; Michael J Dillon, MD, London, UK; Arvind Bagga, MD, New Delhi, India; Karyl Barron, MD, Bethesda, MD, USA; Jean Claude Davin, MD, Amsterdam, The Netherlands; Tomisaku Kawasaki, MD, Tokio, Japan; Carol Lindsley, MD, Kansas City, KS, USA; Ross E Petty, MD, Vancouver, BC, Canada; Anne Marie Prieur, MD, Paris, France; Angelo Ravelli, MD, Genoa, Italy; Patricia Woo, MD, London, UK. Ankara Consensus Conference: Consensus moderator: Nicolino Ruperto, MD, MPH, Genoa, Italy. Moderator assistant: Giovanni Filocamo, MD, Genoa, Italy. Consensus bio-statistician: Angela Pistorio, MD, PhD, Genoa, Italy. PRINTO analysis committee: Nicolino Ruperto, MD, MPH, Genoa, Italy; Seza Ozen, MD, Ankara, Turkey, Principal Investigator; Angela Pistorio, MD, PhD, Genoa, Italy; Angelo Ravelli MD, Genoa, Italy; Alberto Martini MD, Genoa, Italy; Silvia M Iusan, MD, Genoa, Italy; Giovanni Filocamo, MD, Genoa, Italy; Claudia Saad Magalhães, Genoa, Italy. Consensus participants: Paul Brogan, MD, London, UK; David A Cabral, MD, Vancouver, BC, Canada; Ruben Cuttica, MD, Buenos Aires, Argentina; Pavla Dolezalova, MD, Prague, Czech Republic; Raju Khubchandani, MD, Mumbai, India; Daniel Lovell, MD, Cincinnati, OH, USA; Alberto Martini, MD, Genoa, Italy; Kathleen M O'Neil, MD, Oklahoma City, OK, USA; Seza Ozen, MD, Ankara, Turkey, Principal Investigator; Pierre Quartier, MD, Paris, France; Angelo Ravelli, MD, Genoa, France. We are also indebted to all PRINTO/PRES members (97 centres in 36 countries) and to the families for their participation in the study: Albania Anuela Kondi, MD; Argentina Maria Teresa Apaz, MD, Esteban Rodolfo Castell, MD, Ruben Cuttica, MD, Graciela Espada, MD, Stella Maris Garay, MD, Ricardo Russo, MD, Iris Vilca, MD; Austria Jurgen Brunner, MD; Brazil Maria Odete Hilario, MD, Sheila Knupp Feitosa de Oliveira, MD, Claudia Saad Magalhães, MD, Clovis Artur Silva, MD, Flavio Sztajnbok, MD; Bulgaria Dimitrina Mihaylova, MD; Chile Benito Gonzalez, MD, Ximena Norambuena, MD; Croatia Lana Tambic-Bukovac, MD; Czech Republic Pavla Dolezalova, MD; Denmark Troels Herlin, MD, Susan Nielsen, MD; El Salvador Mauricio Alegria, MD; Estonia Chris Pruunsild, MD; France Alexandre Belot, MD, Rolando Cimaz, MD, Djamal Djeddi, MD, Michel Fischbach, MD, Erick Grouteau, MD, Isabelle Kone Paut, MD, Pierre Quartier, MD; Georgia Maka Ioseliani, MD, Karaman Pagava, MD; Germany Frank Dressler, MD, Ivan Foeldvari, MD, Dirk Foell, MD, Hermann Girschick, MD, Gerd Horneff, MD, Rolf Keitzer, MD, Rolf-Michael Kuester, MD, Hartmut Michels, MD, Ralf Trauzeddel, MD, Claudia Sengler MD; Greece Florence Kanakoudi-Tsakalidou, Polyxeni Pratsidou, MD, Olga Vougiouka, MD; India Arvind Bagga, MD, Raju Khubchandani, MD; Iraq Saad Alhasani, MD; Israel Judith Barash, MD, Yackov Berkun, MD, Riva Brik, MD, Tsvia Tauber, MD, Yosef Uziel, MD; Italy Maria Giannina Alpigiani, MD, Giancarlo Barbano, MD, Patrizia Barone, MD, Claudia Bracaglia, MD, Luciana Breda, MD, Antonella Buoncompagni, MD, Giuseppina Calcagno, MD, Luca Cantarini, MD, Rolando Cimaz, MD, Elisabetta Cortis, MD, Gianfranco D'Angelo, MD, Sara Garozzo, MD, Loredana Lepore, MD, Silvia Magni Manzoni, MD, Alma Nunzia Olivieri, MD, Salvatore Scarpato, MD, Achille Stabile, Donato Rigante, MD, Francesco Zulian, MD; Latvia Ingrida Rumba, MD, Valda Stanevicha, MD; Libya Halima Ben Amer, MD; Mexico Adriana Cespedes Cruz, MD, Maria del Rocio Maldonado, MD; Netherlands Esther Hoppenreijs, MD, Marion A J van Rossum, MD, Nico Wulffraat, MD; Peru Amparo Ibanez Estrella, MD; Poland Henryka Mazur-Zielinska, MD; Romania Nicolae Iagaru, MD, Valentin Pop, MD, Calin Lazar, MD; Russia Vyacheslav Chasnyk, MD; Saudi Arabia Sulaiman M Al-Mayouf, MD; Serbia Srdjan Pasic, MD, Gordana Susic, MD; Slovakia Richard Vesely, MD; Slovenia Tadej Avcin, MD; Spain Jordi Anton, MD, María Luz Gamir, MD, Julia Garcia Consuegra, MD, Laura Nuno, MD; Sweden Anders Fasth, MD; Turkey Nuray Aktay Ayaz MD, Esra Baskin, MD, Ilmay Bilge, MD, Zelal Bircan, MD, Faysal Gok, MD, Balahan Makay MD, Seza Ozen, MD, Erbil Unsal, MD; UK Paul Brogan, MD, Kjell Tullus, MD; USA Marisa Klein-Gitelman, MD

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