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Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD
Isabelle Koné-Paut1, Fahrad Shahram2, Martha Darce-Bello1
1Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, APHP, Paris SUD, Le Kremlin Bicêtre, France.
Insights
This study describes Behçet's disease (BD) features in children, proposing a new classification. The research highlights key symptoms and demographic differences in pediatric BD patients for better diagnosis and trials.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Clinical Classification
Background:
- Behçet's disease (BD) is a rare multisystemic vasculitis with variable presentation.
- Accurate classification is crucial for diagnosis and management, especially in pediatric cases.
- Previous classifications lacked validation in large, diverse pediatric cohorts.
Purpose of the Study:
- To characterize the main clinical features of pediatric Behçet's disease.
- To propose the first consensus-based classification for childhood BD.
- To establish criteria for inclusion in future therapeutic trials.
Main Methods:
- Prospective enrollment of 230 pediatric patients across 42 international centers over 66 months.
- Expert consensus group defined minimal inclusion symptoms and classified patients.
- Analysis of concordance with existing international classifications and comparison with control diseases (Eurofever).
Main Results:
- 156 patients (71.2%) were classified with confirmed BD.
- Oral aphthosis was the initial symptom in 81% of patients.
- Demographic and clinical variations observed between European and non-European children, and by sex.
Conclusions:
- A comprehensive description of a large, multinational pediatric BD cohort is provided.
- The first classification system for pediatric Behçet's disease is proposed.
- This classification aids in future research and therapeutic trials for childhood BD.
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.
