Clinical phenotype and long-term damage in paediatric Behçet’s disease: comparative performance of International Study Group (ISG), International Criteria for Behçet's Disease (ICBD) and Paediatric Behçet's Disease (PEDBD) classification criteria in a retrospective endemic cohort
Authors' conclusion
In this endemic paediatric cohort, ICBD demonstrated superior sensitivity, while ISG and PEDBD classified only half of cases. Morbidity was driven by neurovascular events and, less frequently, posterior-segment ocular disease.
Study context
- Population
- Children with clinician-diagnosed paediatric BD (symptom onset ≤18 years), tertiary centre in Türkiye (2020–2025)
- Participants
- 69 consecutive children
- Exposure
- ICBD vs ISG vs PEDBD classification criteria against clinician diagnosis
- Outcome
- Sensitivity of classification criteria vs clinician diagnosis
- Follow-up
- Last visit
Methodological detailssingle-centre, tertiary centre, consecutive patients, endemic cohort
- Single-centre
- Tertiary centre
- Consecutive patients
- Endemic cohort
Main finding
As reported in the abstract
Sensitivity (cases classified) vs clinician diagnosis
Observational data: associations, not effects.
Secondary outcomes
Organ involvement
- Neurovascular involvement (mainly cerebral venous thrombosis)
- 18.8%
- Vascular involvement
- 20.3%
- Ocular involvement
- 17.4%
- Posterior-segment ocular involvement
- 11.6%
Sex-stratified risks
- Boys had higher risks of neurovascular disease
- Girls more often had genital ulcers
Treatment
- Colchicine
- 98.6%
- AZA
- 63.8%
- Anti-TNF
- 21.7%
- Non-adherence
- 11.6%
- Steroid-sparing pathways
Cumulative damage (BODI score)
- Mean BODI score (range 0-3)
- 0.45
- Low to moderate
Safety
- Adverse events
- 15.9%
Original publication
Clinical phenotype and long-term damage in paediatric Behçet’s disease: comparative performance of ISG, ICBD and PEDBD classification criteria in a retrospective endemic cohort. RMD Open. Published 9 March 2026. doi:10.
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