Top

Published in:

01-12-2021 | Colchicine | Research article

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

Authors: Nevin Hammam, Jing Li, Michael Evans, Julia L. Kay, Zara Izadi, Christine Anastasiou, Milena A. Gianfrancesco, Jinoos Yazdany, Gabriela Schmajuk

Published in: Arthritis Research & Therapy | Issue 1/2021

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.

Available only for authorised users

Leonardo NM, McNeil J. Behcet’s disease: is there geographical variation? A review far from the Silk Road. Int J Rheumatol. 2015;2015:1–7.CrossRef

Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM. Epidemiology and clinical characteristics of Behçet’s disease in the US: a population-based study. Arthritis Rheum. 2009;61(5):600–4. https://doi.org/10.1002/art.24423.CrossRefPubMedPubMedCentral

Yazici H, Tüzün Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdoğan H, et al. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis. 1984;43(6):783–9. https://doi.org/10.1136/ard.43.6.783.CrossRefPubMedPubMedCentral

Davari P, Rogers RS, Chan B, Nagler TH, Fazel N. Clinical features of Behçet’s disease: a retrospective chart review of 26 patients. J Dermatol Treat. 2016;27(1):70–4. https://doi.org/10.3109/09546634.2015.1054781.CrossRef

Sibley C, Yazici Y, Tascilar K, Khan N, Bata Y, Yazici H, et al. Behçet syndrome manifestations and activity in the United States versus Turkey — a cross-sectional cohort comparison. J Rheumatol. 2014;41(7):1379–84. https://doi.org/10.3899/jrheum.131227.CrossRefPubMedPubMedCentral

Muruganandam M, Rolle NA, Sibbitt WL, Cook GB, Emil NS, Fangtham M, et al. Characteristics of Behcet’s disease in the American Southwest. Semin Arthritis Rheum. 2019;49(2):296–302. https://doi.org/10.1016/j.semarthrit.2019.03.003.CrossRefPubMed

Shahram F, Mæhlen MT, Akhlaghi M, Davatchi F, Liao YJ, Weyand CM. Geographical variations in ocular and extra-ocular manifestations in Behçet’s disease. Eur J Rheumatol. 2019;6(4):199–206. https://doi.org/10.5152/eurjrheum.2019.18215.CrossRefPubMedPubMedCentral

Yazdany J, Bansback N, Clowse M, Collier D, Law K, Liao KP, et al. Rheumatology informatics system for effectiveness: a national informatics-enabled registry for quality improvement. Arthritis Care Res. 2016;68(12):1866–73. https://doi.org/10.1002/acr.23089.CrossRef

Lenert A, Russell MJ, Segerstrom S, Kim S. Accuracy of US administrative claims codes for the diagnosis of autoinflammatory syndromes. J Clin Rheumatol. 2020.

10.

Deyo RA, Cherkin DC, Ciol MA. Adapting a clinical comorbidity index for use with ICD-9-CM administrative databases. J Clin Epidemiol. 1992;45(6):613–9. https://doi.org/10.1016/0895-4356(92)90133-8.CrossRefPubMed

11.

Gheita TA, El-Latif EA, El-Gazzar II, Samy N, Hammam N, Abdel Noor RA, et al. Behçet’s disease in Egypt: a multicenter nationwide study on 1526 adult patients and review of the literature. Clin Rheumatol. 2019;38(9):2565–75. https://doi.org/10.1007/s10067-019-04570-w.CrossRefPubMed

12.

Yilmaz S, Karadag O, Yazisiz V, Altun B, Gezer M, Karaman M, et al. Systemic involvements and preferred treatments in a large cohort of Behçet’s disease. Rheumatol Int. 2013;33(12):3025–30. https://doi.org/10.1007/s00296-013-2830-0.CrossRefPubMed

13.

Kobayashi T, Kishimoto M, Swearingen CJ, Filopoulos MT, Ohara Y, Tokuda Y, et al. Differences in clinical manifestations, treatment, and concordance rates with two major sets of criteria for Behçet’s syndrome for patients in the US and Japan: data from a large, three-center cohort study. Mod Rheumatol. 2013;23(3):547–53. https://doi.org/10.3109/s10165-012-0696-8.CrossRefPubMed

14.

Mahmoudi M, Aslani S, Meguro A, Akhtari M, Fatahi Y, Mizuki N, et al. A comprehensive overview on the genetics of Behçet’s disease. Int Rev Immunol. 2020:1–64. https://doi.org/10.1080/08830185.2020.1851372.

15.

Kilian NC, Sawalha AH. Behçet’s disease in the United States: a single center descriptive and comparative study. Eur J Rheumatol. 2017;4(4):239–44. https://doi.org/10.5152/eurjrheum.2017.17112.CrossRefPubMedPubMedCentral

16.

Davatchi F, Shahram F, Chams-Davatchi C, Sadeghi Abdollahi B, Shams H, Nadji A, et al. Behcet’s disease: is there a gender influence on clinical manifestations? Int J Rheum Dis. 2012;15(3):306–14. https://doi.org/10.1111/j.1756-185X.2011.01696.x.CrossRefPubMed

17.

Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, et al. Behcet’s disease: from East to West. Clin Rheumatol. 2010;29(8):823–33. https://doi.org/10.1007/s10067-010-1430-6.CrossRefPubMed

18.

Mahr A, Belarbi L, Wechsler B, Jeanneret D, Dhote R, Fain O, et al. Population-based prevalence study of Behcet’s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum. 2008;58(12):3951–9. https://doi.org/10.1002/art.24149.CrossRefPubMed

19.

Ishido T, Horita N, Takeuchi M, Kawagoe T, Shibuya E, Yamane T, et al. Clinical manifestations of Behçet’s disease depending on sex and age: results from Japanese nationwide registration. Rheumatology. 2017;56(11):1918–27. https://doi.org/10.1093/rheumatology/kex285.CrossRefPubMed

20.

Bonitsis NG, Luong Nguyen LB, LaValley MP, Papoutsis N, Altenburg A, Kotter I, et al. Gender-specific differences in Adamantiades-Behcet’s disease manifestations: an analysis of the German registry and meta-analysis of data from the literature. Rheumatol Oxf Engl. 2015;54(1):121–33. https://doi.org/10.1093/rheumatology/keu247.CrossRef

21.

Pathak J, Kho AN, Denny JC. Electronic health records-driven phenotyping: challenges, recent advances, and perspectives. J Am Med Inform Assoc JAMIA. 2013;20(e2):e206–11. https://doi.org/10.1136/amiajnl-2013-002428.CrossRefPubMed

22.

Hammam N, Evans M, Morgan E, Reimold A, Anastasiou C, Kay JL, et al. Treatment of sarcoidosis in U.S. rheumatology practices: data from ACR’s Rheumatology Informatics System for Effectiveness (RISE) Registry. Arthritis Care Res. 2020. https://doi.org/10.1002/acr.24496.

23.

Ananthakrishnan AN, Cai T, Savova G, Cheng S-C, Chen P, Perez RG, et al. Improving case definition of Crohn’s disease and ulcerative colitis in electronic medical records using natural language processing: a novel informatics approach. Inflamm Bowel Dis. 2013;19(7):1411–20. https://doi.org/10.1097/MIB.0b013e31828133fd.CrossRefPubMed

24.

Jamian L, Wheless L, Crofford LJ, Barnado A. Rule-based and machine learning algorithms identify patients with systemic sclerosis accurately in the electronic health record. Arthritis Res Ther. 2019;21(1):305. https://doi.org/10.1186/s13075-019-2092-7.CrossRefPubMedPubMedCentral

25.

Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 Update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808–18. https://doi.org/10.1136/annrheumdis-2018-213225.CrossRefPubMed

26.

Di Scala G, Bettiol A, Cojan RD, Finocchi M, Silvestri E, Emmi G. Efficacy of the anti-IL 17 secukinumab in refractory Behçet’s syndrome: a preliminary study. J Autoimmun. 2019;97:108–13. https://doi.org/10.1016/j.jaut.2018.09.002.CrossRefPubMed

27.

Mirouse A, Barete S, Desbois A-C, Comarmond C, Sène D, Domont F, et al. Long-term outcome of ustekinumab therapy for Behçet’s disease. Arthritis Rheumatol Hoboken NJ. 2019;71(10):1727–32. https://doi.org/10.1002/art.40912.CrossRef

28.

Celgene Corporation. FDA approves OTEZLA® (apremilast) for the treatment of oral ulcers associated with Behçet’s disease. 2019. https://ir.celgene.com/press-releases-archive/press-release-details/2019/FDA-Approves-OTEZLA-apremilast-for-the-Treatment-of-Oral-Ulcers-Associated-with-Behets-Disease/default.aspx. Accessed 19 Oct 2020.

29.

Palestine AG, Kolfenbach JR, Ozzello DJ. Rheumatologists and ophthalmologists differ in treatment decisions for ocular Behçet disease. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis. 2016;22:316–9.

Title: 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
Authors: Nevin Hammam
Jing Li
Michael Evans
Julia L. Kay
Zara Izadi
Christine Anastasiou
Milena A. Gianfrancesco
Jinoos Yazdany
Gabriela Schmajuk
Publication date: 01-12-2021
Publisher: BioMed Central
Keyword: Colchicine
Published in: Arthritis Research & Therapy / Issue 1/2021
Electronic ISSN: 1478-6362
DOI: https://doi.org/10.1186/s13075-021-02615-7

Live Webinar | 27-06-2024 | 18:00 (CEST)

Keynote webinar | Spotlight on medication adherence

Reserve your place

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

WHO estimates that half of all patients worldwide are non-adherent to their prescribed medication. The consequences of poor adherence can be catastrophic, on both the individual and population level.

Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.

Prof. Kevin Dolgin

Prof. Florian Limbourg

Prof. Anoop Chauhan

Developed by: Springer Medicine

Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2021

Exosomal circ-BRWD1 contributes to osteoarthritis development through the modulation of miR-1277/TRAF6 axis

Denosumab alleviates intervertebral disc degeneration adjacent to lumbar fusion by inhibiting endplate osteochondral remodeling and vertebral osteoporosis in ovariectomized rats

Regulation of osteoclastogenesis by mast cell in rheumatoid arthritis

Using a DAS28-CRP-steered treat-to-target strategy does not eliminate subclinical inflammation as assessed by ultrasonography in rheumatoid arthritis patients in longstanding clinical remission

The role of intra-articular neuronal CCR2 receptors in knee joint pain associated with experimental osteoarthritis in mice

Circular RNAs in osteoarthritis: indispensable regulators and novel strategies in clinical implications

Keynote webinar | Spotlight on medication adherence

Live: Thursday 27th June 2024, 18:00-19:30 (CEST)

At a glance: The STEP trials