ArticleRMD open2026
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.
Article in RMD open, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectivesTo compare the sensitivity of International Study Group (ISG, 1990), International Criteria for Behçet's Disease (ICBD, 2014) and the Paediatric Behçet's Disease (PEDBD, 2016) classification criteria in paediatric Behçet's disease (pBD) within an endemic cohort and to assess organ involvement, treatment patterns, outcomes and cumulative damage using the Behçet's Syndrome Overall Damage Index.
methodsWe retrospectively analysed 69 consecutive children with clinician-diagnosed pBD (symptom onset ≤18 years) at a tertiary centre in Türkiye (2020-2025). Classification criteria were applied at the last visit. The primary outcome was sensitivity versus clinician diagnosis; secondary outcomes included sex-stratified risks, therapies/adverse events and cumulative damage.
resultsICBD classified 97.1% of cases, compared with 58.0% for ISG and 53.6% for PEDBD. Neurovascular involvement occurred in 18.8% (mainly cerebral venous thrombosis), vascular in 20.3% and ocular in 17.4% (posterior-segment 11.6%). Boys had higher risks of neurovascular disease, while girls more often had genital ulcers. Treatment followed steroid-sparing pathways (colchicine 98.6%, azathioprine 63.8%, antitumour necrosis factor 21.7%); adverse events occurred in 15.9% and non-adherence in 11.6%. Cumulative damage was low to moderate, with a mean BODI score of 0.45 (range 0-3).
conclusionsIn 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. Findings support an ICBD-anchored approach with co-reporting of ISG/PEDBD for comparability. Low-threshold neuro-ophthalmic evaluation and timely steroid-sparing escalation are critical for sight/central nervous system-threatening disease. Targeted vascular imaging and adherence-focused adolescent care remain priorities.
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