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Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD
  1. Isabelle Koné-Paut1,
  2. Fahrad Shahram2,
  3. Martha Darce-Bello1,
  4. Luca Cantarini3,
  5. Rolando Cimaz4,
  6. Marco Gattorno5,
  7. Jordi Anton6,
  8. Michael Hofer7,
  9. Bouchra Chkirate8,
  10. Kenza Bouayed9,
  11. Ilknur Tugal-Tutkun10,
  12. Jasmin Kuemmerle-Deschner11,
  13. Hélène Agostini12,
  14. Sylvia Federici5,
  15. Armelle Arnoux12,
  16. Celine Piedvache12,
  17. Seza Ozen13
  18. the PEDBD group
    1. 1Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France
    2. 2Rheumatology Research Center, Shariati Hospital, Tehran, Iran
    3. 3Department of Reumatologia, Sienna, Italy
    4. 4Department of Paediatric Rheumatology, A. Meyer Children, Florence, Italy
    5. 5UO Pediatria II, G. Gaslini Scientific Institute, Genoa, Italy
    6. 6Paediatric Rheumatology Unit, Sant Joan de Déu University Hospital, Esplugues de Llobregat, Spain
    7. 7Department of Paediatric Rheumatology, Vaudois University Hospital, Lausanne, Switzerland
    8. 8Internal Medicine and Immunology Clinic, Hassan II University Hospital, Fes, Morocco
    9. 9Department of Paediatrics, IBN Rochd University Hospital, Casablanca, Morocco
    10. 10Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey
    11. 11Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Tübingen, Germany
    12. 12Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France
    13. 13Department of Paediatrics, Hacettepe University, Ankara, Turkey
    1. Correspondence to Professor Isabelle Koné-Paut, Department of Paediatric Rheumatology and Reference Center for Autoinflammatory Diseases, Bicêtre Hospital, APHP, University of Paris SUD, 78 rue du general leclerc, Le Kremlin Bicêtre 94270, France; isabelle.kone-paut{at}


    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).

    Findings 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.

    Interpretation 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.

    • Behcet's disease
    • Epidemiology
    • Fever Syndromes

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