TOPLINE

Prevalence of Behçet's syndrome (BS) in England nearly doubled between 2001 and 2021, while incidence remained stable. Among core clinical features, genital ulceration was associated with the shortest median time to diagnosis compared with uveitis and oral ulceration.

METHODOLOGY

• A retrospective cohort analysis used linked Clinical Practice Research Datalink (CPRD) Aurum and Hospital Episode Statistics (HES) data from England spanning 2001 to 2021 to estimate annual incidence and prevalence of BS.
• A total of 4234 participants were diagnosed with BS during the study period, including 1565 incident cases; in 2021, there were 68 new cases and 1885 prevalent cases among 9,509,280 patients in the linked dataset.
• Researchers identified BS cases using diagnostic codes from either primary care (CPRD) or secondary care (HES) records.
• A nested case-control study within CPRD Aurum compared 1628 cases with a diagnostic code for BS with 11,190 matched control cases without such code to assess the association between a diagnostic code for BS and other inflammatory conditions known to have phenotypical overlap (genital ulceration, uveitis, oral ulceration, folliculitis, and others).

TAKEAWAY

• The incidence of BS was stable from 0.78 per 100,000 person-years in 2001 to 0.75 in 2021; the prevalence rose year on year, from 8.62 per 100,000 in 2001 to 19.80 in 2021.
• Prevalence was highest in the 31-40 years age group and lowest in those aged 0-16 years and 81+ years; both prevalence and incidence were higher in women and in individuals of mixed race or 'other' ethnicity.
• Genital ulceration showed the strongest association with BS (adjusted odds ratio [aOR], 155.08; 95% CI, 77.90-367.40), followed by uveitis (aOR, 43.00; 95% CI, 30.39-62.59) and oral ulceration (aOR, 24.73; 95% CI, 20.14-30.54).
• Development of genital ulceration as the first clinical feature was associated with the shortest time to a confirmed BS diagnosis (240 days), followed by uveitis (377.5 days) and oral ulceration (558 days).

IN PRACTICE

"Confirmation of a coded BS diagnosis following appearance of key phenotypical manifestations is often delayed, likely due to the complex and overlapping multisystemic nature of the condition. Improving recognition of the spectrum of BS manifestations and refining classification criteria performance in non-endemic settings may improve disease recognition and reduce diagnostic delay," the authors of the study wrote.

SOURCE

The study was led by Priyanka Chandratre, The Ottawa Hospital Research Institute in Ottawa, Ontario, Canada. It was published online on July 27 in RMD Open.

LIMITATIONS

Case ascertainment relied solely on diagnostic codes without direct validation against classification criteria, specialist confirmation, or multidisciplinary team review. Ethnicity and migration status were incompletely recorded in primary care databases. The extremely high odds ratios for core BS features reflect circular reasoning to some degree, as these manifestations are part of the diagnostic criteria themselves.

DISCLOSURES

The study was supported by Behcets UK. The authors reported no relevant conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.