Background/Purpose: Behçet’s disease (BD), a rare multisystem vasculitis, is prevalent among Middle Eastern populations but remains rare in North America. Data for patients with BD in the U.S. are based on cohorts from single centers and limited by small sample sizes. Therefore, we sought to characterize BD in the U.S., analyzing patient characteristics and medication use across the RISE registry.   We also compared the characteristics of U.S. patients with those from the Egyptian College of Rheumatology (ECR) registry, which represents one of the largest cohorts of BD patients to date (Gheita TA, et al. Clin Rheumatol. 2019).

Methods: We conducted a cross-sectional study using data from the RISE Registry (2014-2018). RISE contains patient data collected during routine clinical care through EHRs of participating rheumatology practices. As of 2018, RISE held validated data from 1,113 providers in 226 practices, representing ~32% of the US clinical rheumatology workforce. Patients aged ≥ 18 years with ≥ 1 diagnosis codes (ICD-9 or ICD-10) for BD were included. Sociodemographic, laboratory, and treatment information was extracted when available. The ECR BD registry, a population-based multicenter cohort, included adults aged ≥ 18 years who fulfilled the 2014 International Study Group diagnostic criteria for BD who presented to one of the included rheumatology centers during 2017-2018. Patients’ demographic, clinical, and laboratory data in the ECR were collected and entered into a standardized data abstraction form. Data were summarized with descriptive statistics and comparisons were performed using t-tests and chi-square tests, as appropriate.

Results: A total of 1323 subjects with BD from the RISE registry were included. The mean ± SD age was 48.7 ± 16.3 years, female to male ratio was 3.8:1, with 66.7% being White (Table 1). The most frequently used medications included glucocorticoids (67.6%) and colchicine (55.0%). Infliximab and adalimumab were the most commonly used biologics (14.5% and 14.1%, respectively); 3.2% of patients used apremilast.

In comparison to the ECR cohort, the RISE cohort had more women (49.0% in RISE vs. 27.8% in ECR, p < 0.001, Table 2) and patients were older (mean age (SD); 48.7 (16.3) in RISE vs. 35.7 (9.84) in ECR, p < 0.001). There were also significant differences in medication use, with methotrexate and TNFi used more commonly in RISE (21.8% and 37.1%) compared to the ECR (7.2% and 8.3%, p < 0.001) whereas colchicine, glucocorticoids, cyclosporine and cyclophosphamide had more widespread use in the ECR cohort.

Conclusion: We report on the largest population-based BD cohort in the U.S. using the RISE registry. Our findings suggest that U.S. patients with BD are predominantly female, which is in contrast to cohorts in the Middle East. Differences in medication use may be due to practice patterns or differences in the manifestations of BD. Further research is needed to explore the reasons for the increased prevalence of BD among women in the U.S. and its possible impact on disease severity and management.

Disclaimer:

This data was supported by the ACR’s RISE Registry. However, the views expressed represent those of the authors, not necessarily those of the ACR.

Keywords:

Behçet’s disease, RISE, ECR, medication

Table 1: Characteristics of patients with Behçet’s disease in the RISE registry

Table 2: Comparison of BD characteristics between RISE and Egyptian college of rheumatology cohorts

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/epidemiology-and-treatment-of-behcets-disease-insights-from-the-rheumatology-informatics-system-for-effectiveness-rise-registry/