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, cilt.12, sa.1, 2026 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 12 Sayı: 1
- Basım Tarihi: 2026
- Doi Numarası: 10.1136/rmdopen-2025-006514
- Dergi Adı: RMD OPEN
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE, Directory of Open Access Journals, Health Research Premium Collection (ProQuest)
- Ondokuz Mayıs Üniversitesi Adresli: Evet
Özet
Objectives To compare the sensitivity of International Study Group (ISG, 1990), International Criteria for Beh & ccedil;et's Disease (ICBD, 2014) and the Paediatric Beh & ccedil;et's Disease (PEDBD, 2016) classification criteria in paediatric Beh & ccedil;et's disease (pBD) within an endemic cohort and to assess organ involvement, treatment patterns, outcomes and cumulative damage using the Beh & ccedil;et's Syndrome Overall Damage Index.Methods We retrospectively analysed 69 consecutive children with clinician-diagnosed pBD (symptom onset <= 18 years) at a tertiary centre in T & uuml;rkiye (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.Results ICBD 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).Conclusions 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. 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.