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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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