Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD.
- Koné-Paut I Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
- Shahram F Rheumatology Research Center, Shariati Hospital, Tehran, Iran.
- Darce-Bello M Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
- Cantarini L Department of Reumatologia, Sienna, Italy.
- Cimaz R Department of Paediatric Rheumatology, A. Meyer Children, Florence, Italy.
- Gattorno M UO Pediatria II, G. Gaslini Scientific Institute, Genoa, Italy.
- Anton J Paediatric Rheumatology Unit, Sant Joan de Déu University Hospital, Esplugues de Llobregat, Spain.
- Hofer M Department of Paediatric Rheumatology, Vaudois University Hospital, Lausanne, Switzerland.
- Chkirate B Internal Medicine and Immunology Clinic, Hassan II University Hospital, Fes, Morocco.
- Bouayed K Department of Paediatrics, IBN Rochd University Hospital, Casablanca, Morocco.
- Tugal-Tutkun I Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
- Kuemmerle-Deschner J Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tübingen, Tübingen, Germany.
- Agostini H Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
- Federici S UO Pediatria II, G. Gaslini Scientific Institute, Genoa, Italy.
- Arnoux A Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
- Piedvache C Clinical Research Unit, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
- Ozen S Department of Paediatrics, Hacettepe University, Ankara, Turkey.
- 2015-12-25
Published in:
- Annals of the rheumatic diseases. - 2016
Behcet's disease
Epidemiology
Fever Syndromes
Adolescent
Age of Onset
Behcet Syndrome
Child
Consensus
Female
Humans
Male
Prospective Studies
Symptom Assessment
English
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.
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.
- Language
-
- English
- Open access status
- green
- Identifiers
-
- DOI 10.1136/annrheumdis-2015-208491
- PMID 26698843
- Persistent URL
- https://folia.unifr.ch/global/documents/123078
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