Phenotypic subgroups and classification criteria performance in paediatric Behçet’s disease: insights on systemic involvement
Authors' conclusion
Early-onset BD shows similar clinical profiles to later-onset disease despite a tendency towards longer diagnostic delay. Current criteria insufficiently detect neurologic and vascular phenotypes
Study context
- Population
- Paediatric behçet's disease (BD) patients from a tertiary centre
- Participants
- 38 patients
- Exposure
- Early-onset vs late-onset paediatric BD
- Outcome
- Clinical characteristics by age at onset and factors contributing to diagnostic delay
Methodological detailssingle tertiary centre
- Single tertiary centre
Main finding
As reported in the abstract
- Early-onset patients had younger symptom onset but a diagnostic delay similar to late-onset cases
- Clinical manifestations, laboratory findings and treatment approaches comparable between groups
- ISG, ICBD and PEDBD scores showed no differences and strong inter-correlations
Observational data: associations, not effects.
Further findings
Classification criteria performance and phenotypes
- Mucocutaneous features (genital ulcers, oral aphthae, skin lesions) significantly less common in ICBD-negative patients
- PEDBD positivity corresponded to mucocutaneous-dominant phenotypes
- CNS and vascular involvement frequently co-occurred and were associated with fewer mucocutaneous findings
- None met ISG criteria; only one-third met ICBD
- No significant associations for ocular, joint or febrile symptoms
- Multivariate analysis not feasible due to small subgroup sizes
Original publication
Phenotypic subgroups and classification criteria performance in paediatric Behçet’s disease: insights on systemic involvement. Rheumatology (Oxford). Published 29 November 2025. doi:10.
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