Behçet Disease Shows Higher Prevalence Among Women in the US

A cross-sectional study of data from the RISE rheumatology registry indicates that despite the male-predominant presentation of Behcet disease in endemic regions, such as Turkey and Iran, this rare form of multisystem vasculitis is more prevalent among women in the United States.

The authors noted that Behcet disease remains rare in the US, where the estimated prevalence ranges from 0.33 to 5.2 people per 100,000 population, compared to a prevalence of up to 370 per 100,000 in Turkey, where the condition is endemic. However, they commented, “The prevalence of BD in the U.S. is increasing, which may be due to increased disease recognition and immigration from endemic areas; however, robust epidemiologic data about BD in the U.S. is scarce.”

Researchers delved into the RISE national rheumatology registry, which includes data collected through electronic health records on patients treated during routine outpatient clinical care at 226 participating rheumatology practices across the US. These data encompass demographics, diagnoses, procedures, medications, laboratory test results, and vital signs. The study incorporated information concerning patients aged 18 years and older with a diagnosis of BD and compared the data to those from published studies of patients with BD from endemic areas.

The investigators included data on 1323 patients with BD from the RISE registry with a mean age of 48.7 ± 16.3 years. The female to male ratio was 3.8:1—virtually the reverse of the ratio between men and women in Turkey—and 66.7% were White.

“The current study represents the largest dataset of U.S. BD patients reported to date. We confirmed the female predominance in the U.S. and found that a substantial proportion of BD patients were taking a biologic medication,” the authors wrote. In comparison to patients in endemic regions, American  patients with BD were older and received more biological medications, “which raises the possibility that there are a variety of epidemiologic and clinical differences in disease between patients in the U.S. and those from endemic areas. Future studies examining clinical manifestations among large numbers of U.S. BD patients are needed.”

--Rebecca Mashaw

Reference:

Hammam N, Li J, Evans M. et al. Epidemiology and treatment of Behçet’s disease in the USA: insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry with a comparison with other published cohorts from endemic regions. Arthritis Res Ther. Published online August 30, 2021. https://doi.org/10.1186/s13075-021-02615-7