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

Abstract

Background Behçet’s disease (BD), a chronic systemic vasculitis, has distinct geographical and ethnic variation. Data regarding the epidemiology of patients with BD in the U.S. are limited; therefore, we sought to describe BD patient characteristics and medication use in the U.S., and compared them with data from patients from endemic regions. Methods We conducted a cross-sectional study using data from the RISE registry (2014–2018). Patients aged ≥ 18 years with BD were included. Sociodemographic and treatment information was extracted. We compared patients from the RISE registry to data from other published studies of patients with BD from endemic areas. Results One thousand three hundred twenty-three subjects with BD from the RISE registry were included. Mean age was 48.7 ± 16.3 years, female to male ratio was 3.8:1, and 66.7% were White. The most frequently used medications included glucocorticoids (67.6%) and colchicine (55.0%). Infliximab and adalimumab were the most used biologics (14.5% and 14.1%, respectively); 3.2% of patients used apremilast. The RISE registry had more women (79.3%), and patients were older compared to previously published BD studies from endemic areas. Methotrexate and TNFi were more commonly reported in RISE (21.8% and 29.4%) compared to studies from Egypt and Turkey. Colchicine, cyclosporine, and cyclophosphamide were more commonly used in cohorts from Egypt, Turkey, and Iran. Conclusions Findings from the largest BD dataset in the U.S. suggest that BD patients are predominantly female. Further research is needed to explore the reasons for the higher prevalence of BD among women in the U.S. and its possible impact on disease severity and management.

A patient-driven registry on Behçet’s disease: the AIDA for patients pilot project

“…Indeed, the M/F ratio in BD subjects varies from 0.8 to 2.4 in the largest cohorts from the literature, with a similar frequency among men and women in most part of the world or a slight preference for males (7)(8)(9)(10)(11)(12). On the other hand, a recent epidemiology study on 1,323 patients from the US reported a 0.3 M/F ratio (13). In this context, given the methodology of this study, we cannot exclude a bias caused by the multiple recruitment channels with a preference for the spontaneous enrolment versus the traditional hospital-based one.…”

Patient Experiences and Challenges in the Management of Autoinflammatory Diseases—Data from the International FMF & AID Global Association Survey

“…Variants associated with AIDs are included in the 2022 classification of human inborn errors of immunity [ 18 ], many of them caused by monogenic germline or somatic mutations, and include 485 distinct disorders categorized into autoimmune, autoinflammatory, allergic, and malignant phenotypes [ 19 ]. The most well-known AID is familial Mediterranean fever (FMF), which is caused by variants in the MEFV gene [ 20 ]. It is known to be highly prevalent in the eastern Mediterranean region.…”

Epidemiology of systemic vasculitis

“…The well known epidemiologic features of Behçet syndrome (BS) are its high prevalence along the ancient Silk Route, increasing frequency from North to South in Europe, usual disease onset during the second decade of life, and similar frequency among men and women, but more severe disease among men. A recent study that explored demographic characteristics and prescription data in a US database between 2014 and 2018 showed that among 1324 BS patients 79% were women [32 ▪▪ ]. Glucocorticoid and biologic prescription rates were similar, suggesting similar disease severity between men and women.…”