Vasculitis & polymyalgia rheumatica

Behçet’s syndrome epidemiology in England: a real-world retrospective cohort analysis with a nested case-control study using linked UK primary and secondary care data

RMD OpenPublished Retrospective cohort study with a nested case-control study

Authors' conclusion

BS prevalence appears to increase over time in the UK, potentially linked to changing population demographics and migration, and to performance characteristics of the 2014 International Criteria for Behçet's Disease classification criteria in low-prevalence settings.

Study context

Population
Patients with Behçet's syndrome in England, Clinical Practice Research Datalink (CPRD) Aurum and linked Hospital Episode Statistics (HES), 2001–20211,565 incident behçet's syndrome (BS) cases
Participants
1,628 vs 11,190
Comparison
BS cases vs matched controls; incidence and prevalence of BS
  • BSn = 1,628
  • Controlsn = 11,190reference
Outcome
Incidence and prevalence of BS
Methodological detailslinked primary and secondary care data (CPRD Aurum and HES), matched case-control design, Case ascertainment relied on diagnostic coding without direct validation against classification criteria
  • Linked primary and secondary care data (CPRD Aurum and HES)
  • Matched case-control design
  • Case ascertainment relied on diagnostic coding without direct validation against classification criteria

Main finding

As reported in the abstract

Incident BS cases in study interval

1565

Incidence rate in 2021 (per 100 000 person years at risk)

0.75/100 000

Prevalence rose from (per 100 000 person years)

8.62

Prevalence rose to (per 100 000 person years)

19.80

Observational data: associations, not effects.

Further findings

OutcomeEstimate
Clinical triad (genital ulceration, uveitis, oral ulceration), nested case-controlBS vs Controls · genital ulceration · matched case-controlaOR 155.08
Clinical triad (genital ulceration, uveitis, oral ulceration), nested case-controlBS vs Controls · uveitis · matched case-controlaOR 43.00
Clinical triad (genital ulceration, uveitis, oral ulceration), nested case-controlBS vs Controls · oral ulceration · matched case-controlaOR 24.73
Crohn's disease, nested case-controlMatched case-controlOR 5.69

BS prevalence over the study interval · 2001–2021

Prevalence rose from (per 100 000 person years)
8.62
Prevalence rose to (per 100 000 person years)
19.80

Time from first clinical code of genital ulceration to BS diagnosis (shortest interval of manifestations)

Median interval to diagnosis after first genital ulceration code
240 days; IQR 63-745

Original publication

Behçet’s syndrome epidemiology in England: a real-world retrospective cohort analysis with a nested case-control study using linked UK primary and secondary care data. RMD Open. Published 27 July 2026. doi:10.1136/rmdopen-2025-006594

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