title: Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD.
abstract: BACKGROUND
  We aimed to describe the main features of Behçet's disease (BD) in children in the largest prospective cohort to date and to propose a classification.
  
  
  METHODS
  An international expert consensus group was formed to define a data set of minimal symptoms for the inclusion of patients. Patients were entered prospectively during 66 months. Experts classified patients on a consensus basis. The concordance of two international classifications was analysed in confirmed patients with BD. Comparisons of subgroups of patients helped define consensus criteria. BD-associated clinical manifestations were also investigated in three control diseases extracted from an independent data set (Eurofever).
  
  
  RESULTS
  In total, 42 centres from 12 countries included 230 patients; data for 219 (M/F ratio=1) could be analysed. The experts classified 156 patients (71.2%) as having confirmed BD. Males more often than females showed cutaneous, ocular and vascular symptoms and females more often genital aphthosis. Age at disease onset and skin and vascular involvement were lower for European than non-European children. Oral aphthosis was the presenting sign for 81% (179/219) of patients. The mean delay to the second symptom was 2.9±2.2 years. International classifications were not concordant with the expert classification. Our paediatric classification contains six categories, a minimum of three signs (each in a distinct category) defining paediatric BD. Three clinical signs discriminated our cohort from the Eurofever cohorts.
  
  
  CONCLUSIONS
  We present a comprehensive description of a large cohort of patients from both European and non-European countries and propose the first classification of paediatric BD for future therapeutic trials.
authors.affiliation: Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Koné-Paut
authors.affiliation: Rheumatology Research Center, Shariati Hospital, Tehran, Iran.
authors.forename: Fahrad
authors.initials: F
authors.lastname: Shahram
authors.affiliation: Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
authors.forename: Martha
authors.initials: M
authors.lastname: Darce-Bello
authors.affiliation: Department of Reumatologia, Sienna, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.affiliation: Department of Paediatric Rheumatology, A. Meyer Children, Florence, Italy.
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
authors.affiliation: UO Pediatria II, G. Gaslini Scientific Institute, Genoa, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
authors.affiliation: Paediatric Rheumatology Unit, Sant Joan de Déu University Hospital, Esplugues de Llobregat, Spain.
authors.forename: Jordi
authors.initials: J
authors.lastname: Anton
authors.affiliation: Department of Paediatric Rheumatology, Vaudois University Hospital, Lausanne, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Hofer
authors.affiliation: Internal Medicine and Immunology Clinic, Hassan II University Hospital, Fes, Morocco.
authors.forename: Bouchra
authors.initials: B
authors.lastname: Chkirate
authors.affiliation: Department of Paediatrics, IBN Rochd University Hospital, Casablanca, Morocco.
authors.forename: Kenza
authors.initials: K
authors.lastname: Bouayed
authors.affiliation: Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
authors.forename: Ilknur
authors.initials: I
authors.lastname: Tugal-Tutkun
authors.affiliation: Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Tübingen, Germany.
authors.forename: Jasmin
authors.initials: J
authors.lastname: Kuemmerle-Deschner
authors.affiliation: Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Agostini
authors.affiliation: UO Pediatria II, G. Gaslini Scientific Institute, Genoa, Italy.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Federici
authors.affiliation: Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
authors.forename: Armelle
authors.initials: A
authors.lastname: Arnoux
authors.affiliation: Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
authors.forename: Celine
authors.initials: C
authors.lastname: Piedvache
authors.affiliation: Department of Paediatrics, Hacettepe University, Ankara, Turkey.
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001528:Behcet Syndrome / Q000145:classification* / Q000175:diagnosis; D002648:Child; D032921:Consensus; D005260:Female; D006801:Humans; D008297:Male; D011446:Prospective Studies; D063189:Symptom Assessment / Q000145:classification*
keywords: Behcet's disease; Epidemiology; Fever Syndromes