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Published March 28, 2022 | Version v1
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33. An update on the epidemiology of Behçet's disease in southern Sweden

  • 1. 1Department of Clinical Sciences - Rheumatology, Lund University, Lund, Sweden,
  • 2. 1Department of Clinical Sciences - Rheumatology, Lund University, Lund,
  • 3. 2Vasculitis Research Centre, Hacettepe University, Ankara, Turkey
  • 4. 1Department of Clinical Sciences - Rheumatology, Lund University, Lund, ; 3Department of Medicine, University of Cambridge, Cambridge, United Kingdom

Description

Background: Behçet’s disease (BD) is a variable-vessel vasculitis with a distinct geographical distribution of high prevalence along the ancient Silk Route, extending from eastern Asia to the Mediterranean. The prevalence and incidence rate in southern Sweden have previously been estimated to 4.9 per 100,000 adults and 0.2 per 100,000 person-years, respectively. Since then, the proportion of the population of non-Swedish ancestry has increased in southern Sweden. Furthermore, immigration patterns might have an increasing influence on epidemiology of BD. This study is aimed to update the epidemiology of BD in southern Sweden.

 

Methods: The study area was the county of Skåne with an adult (≥18 years) population of 1 080 664 on 31st of December 2019. Patients assigned to the ICD-10 code M35.2 between 1998 and 2019 were retrospectively identified by search in population-based data base. Patients fulfilling the International Study Group diagnostic criteria for BD were included. The point prevalence per 100 000 adults was estimated on the 31st of December 2019. Incidence rate of BD was estimated per 100 000 adult person-years. Non-Swedish ancestry was defined as a person being born outside of Sweden or with two parents born outside of Sweden. A case was considered incident when the diagnosis was made within Skåne with a minimum of 3 years of residence in Sweden prior to diagnosis. 

 

Results: 101 patients fulfilling the diagnostic criteria for BD were identified (61 men and 40 women). The point prevalence was 9.1/100 000 adults (95% CI 7.3, 10.9) and higher among the population of non-Swedish ancestry (19.3 vs. 4.8/100 000, p = 0.001) and higher among men (11.2 vs 7.0/100 000, p =0.027). There were 58 adult incident cases diagnosed between 1998 and 2019. The incidence rate was 0.3/100 000 person-years (95% CI 0.2,0.3) and was higher among the population of non-Swedish ancestry 0.7 vs. 0.2/100 000, p =0.001). There was no significant increase in incidence rate of BD over time (Table 1). All patients presented with oral ulcerations, 90% with genital ulcerations, 84% with skin lesions and 53% with eye disease. 18% experienced thromboembolic events, 14 men and 4 women.

 

Conclusions: The prevalence of BD in southern Sweden has increased since last estimated. The incidence rate of BD has not increased significantly during the study period, despite an increased proportion of the population being of non-Swedish ancestry. Although the study was in a population-based setting the low sample size is a limitation.

 

Disclosures: None.

 

 

 

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