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September 16, 2021
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US patients with Behçet's disease mostly female, with more biologic use vs. endemic areas

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The largest dataset of U.S. patients with Behçet’s disease to date shows that nearly 80% are women, according to researchers who published their findings in Arthritis Research & Therapy.

Perspective from Barbara Kienzle, BSN, RN

The researchers additionally found that patients with Behçet’s disease in the United States are older and receive more biological drugs compared with those in endemic regions, such as Egypt, Iran, Japan and Turkey.

The largest dataset of U.S. patients with Behçet’s disease to date shows that nearly 80% are women, according to researchers. Data derived from Hammam N, et al. Arthritis Res Ther. 2021;doi:10.1186/s13075-021-02615-7.
The largest dataset of U.S. patients with Behçet’s disease to date shows that nearly 80% are women, according to researchers. Data derived from Hammam N, et al. Arthritis Res Ther. 2021;doi:10.1186/s13075-021-02615-7.

“The prevalence of the disease, the frequency of specific clinical findings, and the mortality rate have distinct geographical and ethnic variation: [Behçet’s disease (BD)] prevalence is higher in the Middle East and East Asia but remains rare in North America,” Gabriela Schmajuk, MD, of the University of California, San Francisco, and colleagues wrote. “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.

“While descriptive cohort studies of BD in the U.S. exist, they are from single centers, limited by small sample sizes, and have not reported data from multiple racial or ethnic groups,” they added. “Furthermore, previous studies comparing the characteristics of patients with BD from the northeastern U.S. with patients from Turkey and Iran raised the possibility that BD in U.S. patients may have unique features compared to typical BD populations. However, to date, no national studies of BD in the U.S. have been published.”

Gabriela Schmajuk

To assess the characteristics and medication use of U.S. patients with Behçet’s disease, compared with those from endemic regions, Schmajuk and colleagues conducted a cross-sectional study of data from the RISE registry, a national rheumatology registry of all patients seen during routine outpatient care based on electronic health records provided by participating U.S. practices. As of 2018, the dataset included records from 1,113 providers at 226 practices, representing more than 30% of the total U.S. clinical rheumatology workforce.

For their study, the researchers included a total of 1,323 adult patients with Behçet’s disease from the RISE registry. Sociodemographic and treatment data were extracted and compared with those from published studies of patients with the disease from endemic areas. These included the Egyptian College of Rheumatology Registry, with 1,526 participants; as well as studies from Turkey, with 682 participants; Iran, with 163; and Japan, with 135.

According to the researchers, data from the RISE registry demonstrated a mean age of 48.7 ± 16.3 years, a female-to-male ratio of 3.8:1, and that 66.7% of patients were white. The most common treatments were glucocorticoids, at 67.6%, and colchicine, at 55%. Meanwhile, infliximab (Remicade, Janssen) and adalimumab (Humira, AbbVie) were the most common biologics — at 14.5% and 14.1%, respectively — and 3.2% of patients used apremilast (Otezla, Amgen).

In all, 79.3% of patients in the RISE registry were women, compared with 27.8% in the Egyptian registry, 16.6% in the Turkish cohort, 38.7% in the Iranian study, and 57.8% in the Japanese cohort.

Additionally, U.S. patients were older compared to those in endemic areas, with mean age of 48.7 (SD = 16.3) years in RISE versus 35.7 (9.84) years in the Egyptian registry and 33.9 (9.9) years in the Turkish study. Methotrexate and TNF inhibitors were more common in RISE — at 21.8% and 29.4%, respectively — compared to patients from Egypt — 7.2% and 8.3%, respectively — and Turkey. Meanwhile, colchicine, cyclosporine and cyclophosphamide were common in the cohorts from Egypt, Turkey and Iran.

“This is the largest U.S. cohort of patients with Behçet’s,” Schmajuk told Healio Rheumatology. “We found that among patients seeing rheumatologist, the majority of them are women. This confirms previous reports from smaller studies. Nearly a third of patients are being treated with TNF inhibitors, which is much higher than in studies reported from endemic regions.”

“In terms of clinical significance: In comparison to patients in endemic regions, U.S. patients with Behçet’s were older and received more biologic 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,” she added. “Future studies examining clinical manifestations among large numbers of U.S. Behçet’s disease patients are needed.”