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Behçet’s Disease, also known as Behçet’s Syndrome, is a rare, chronic, multisystem inflammatory disease. Behçet’s Disease is a form of variable-vessel vasculitis, affecting both arteries and veins of all sizes. The most common manifestation is mucocutaneous lesions accompanied by lesions in other organ systems.1-4
Genital Ulcers
up to 80%
+
Genital Ulcers
up to 80%
Including: Scrotal lesions, vulval lesions, epididymitis.
Specialist: Urologist, gynecologist.
View images of genital ulcers here.
Ocular Disease
up to 50%
+
Ocular Disease
up to 50%
Including: Anterior uveitis, relapsing hypopyon, vitritis, retinal infiltrates, retinal vasculitis, retinal vascular occlusion.
Specialist: Ophthalmologist.
View an image of ocular disease here.
Vascular
up to 16%
+
Vascular
up to 16%
Including: Ischemic heart disease; cardiac, pulmonary, and peripheral vessel aneurysms; deep venous occlusions.
Specialist: Cardiologist, pulmonologist, vascular surgeon.
Gastrointestinal
0%-25%
+
Gastrointestinal
0%-25%
Including: Aphthous ulcers in any part of the GI tract.
Specialist: Primary care physician, gastroenterologist.
Oral Ulcers
up to 98%
+
Oral Ulcers
up to 98%
Including: Aphthous ulcers in the mouth and/or throat.
Specialist: Primary care physician, dentist, oral surgeon.
View images of oral ulcers here.
Skin Lesions
up to 80%
+
Skin Lesions
up to 80%
Including: Folliculitis, erythema nodosum, pathergy response.
Specialist: Dermatologist.
View images of skin lesions here.
Joints
up to 45%
+
Joints
up to 45%
Including: Non-obliterative arthritis, usually in large joints.
Specialist: Rheumatologist.
Neurological
5%-25%
+
Neurological
5%-25%
Including: Parenchymal disease, cerebral sinus thrombosis.
Specialist: Neurologist.
The manifestations of Behçet’s are recurring and remitting and may not occur simultaneously.3 They may present as seemingly isolated conditions and are often treated by different medical specialists depending on the system affected. For example, oral ulcers, the most common onset manifestation, are often treated by a dentist or a general practitioner. Similarly, a dermatologist will typically see and treat skin manifestations, while a gynecologist or urologist may treat genital ulcers.2,3,7
Suspect Behçet’s disease?
Refer to a rheumatologist to confirm a diagnosis
Andreas Altenburg, Nadine El-Haj, Christiana Micheli, Marion Puttkammer, Mohammed Badawy Abdel-Naser, Christos C. Zouboulis. The Treatment of Chronic Recurrent Oral Aphthous Ulcers. Deutsches Ärzteblatt International. 2014;111:665-673. Figures used with kind permission from Springer-Verlag, Heidelberg.
Ilknur Tugal-Tutkun. Behçet's Uveitis. Middle East African Journal of Ophthalmology, 2009 Oct-Dec;16(4):219-224.
Reproduced from Postgraduate Medical Journal. V Kontogiannis, R J Powell, Volume 76, issue 900, figures 1-8, year 2000 from BMJ Publishing Group Ltd.
Copyright Mediscan/Visuals Unlimited, Inc.
Reproduced from Postgraduate Medical Journal. V Kontogiannis, R J Powell, Volume 76, issue 900, figures 1-8, year 2000 from BMJ Publishing Group Ltd.
Reproduced from Postgraduate Medical Journal, V Kontogiannis, R J Powell, Volume 76, issue 900, figures 1-8, year 2000 from BMJ Publishing Group Ltd.
Andreas Altenburg, Nadine El-Haj, Christiana Micheli, Marion Puttkammer, Mohammed Badawy Abdel-Naser, Christos C. Zouboulis. The Treatment of Chronic Recurrent Oral Aphthous Ulcers. Deutsches Ärzteblatt International. 2014;111:665-673. Figures used with kind permission from Springer-Verlag, Heidelberg.
Andreas Altenburg, Nadine El-Haj, Christiana Micheli, Marion Puttkammer, Mohammed Badawy Abdel-Naser, Christos C. Zouboulis. The Treatment of Chronic Recurrent Oral Aphthous Ulcers. Deutsches Ärzteblatt International. 2014;111:665-673. Figures used with kind permission from Springer-Verlag, Heidelberg.
Patients (%)
The manifestations of Behçet's Disease (BD) vary widely in type and severity, with the potential to impact multiple organ systems. However, nearly every person with Behçet’s has frequent, recurrent oral ulcers and typically multiple ulcers at once.9 These ulcers are painful and can be refractory to treatment.3,10 Often the first manifestation of Behçet’s, oral ulcers, contribute substantially to diminished quality of life (QoL) as a result of their debilitating impact on daily functions such as chewing, swallowing, and speaking.11-14 Recurrent oral ulcers in Behçet’s tend to crop and scar.3,15
A systematic literature review of over 2500 citations and >21,000 patients with BD described, confirmed that most common manifestation of Behçet’s disease is oral ulcers, with 19 of 27 studies reporting 100% prevalence. One study reported an average of 12.7 oral ulcers per patient per year; in another study, OU were the only manifestation currently or previously experienced by all patients. Frequently reported symptoms included fatigue/sleep-related issues (70%), pain (59%), and headache (58%); skin lesions were also common (58%). Several comorbidities, eg, emotional issues (24%-44%), fibromyalgia (8%-24%), and hearing loss (10%-59%), were significantly increased in patients with BD vs healthy controls. Patients with BD have similar or significantly worse quality of life compared with other chronic inflammatory conditions (eg, rheumatoid arthritis).16
In addition to oral ulcers, many people experience other painful manifestations such as arthritis, genital ulcers, and skin lesions. The pain caused by these manifestations can also negatively impact QoL, with visible manifestations also affecting body image.11
bStudy of 2656 citations and posters examining real-world Behçet's Disease in patients with oral ulcers, and other manifestations of disease.
No single underlying cause of Behçet's Disease has been identified. It is thought that genetic predisposition and environmental triggers may work together to activate an autoimmune cascade that leads to Behçet's Disease.3 The exact mechanism of the inflammatory changes in Behçet's Disease remains unknown.4
HLA, human leukocyte antigen; IFN, interferon; IL, interleukin; TNF, tumor necrosis factor.
Currently, there are limited approved therapies available to treat Behçet’s Disease.1,7 Symptom management strategies involve anti-inflammatory and immunosuppressive agents. Long-term treatment with some agents may be limited by their side effect profile.3
Due to the involvement of different organ systems, guidelines recommend a multidisciplinary approach to managing the manifestations of Behçet’s Disease.1 While a rheumatologist may be engaged to manage inflammation and will lead the multidisciplinary team, a specialist in a specific area, such as a dermatologist or a dentist, may offer symptom management for skin lesions or oral ulcers, respectively.7
Help raise awareness of the
challenges of Behçet’s Disease and reduce the delay in diagnosis
The most common manifestations of Behçet’s Disease, mucocutaneous lesions, frequently contribute to misdiagnoses.3,4 Sharing the below information via social media will help raise awareness of Behçet’s Disease and may even prevent a delay in its diagnosis.
1. Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. 2018;77(6):808-818.
2. Barnes CG. History and diagnosis. In: Yazıcı Y, Yazıcı H, eds. Behçet’s Syndrome. New York, NY: Springer; 2010:7-33.
3. Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P. Behçet’s disease physiopathology: a contemporary review. Auto Immun Highlights. 2016;7(1):4.
4. Leonardo NM, McNeil J. Behçet's disease: is there geographical variation? A review far from the Silk Road. Int J Rheumatol. 2015;2015:945262.
5. Verity DH, Wallace GR, Vaughan RW, Stanford MR. Behçet’s disease: from Hippocrates to the third millennium. Br J Ophthalmol. 2003;87(9):1175-1183.
6. Demirkesen C, Öz B, Göksel S. Behçet’s disease: pathology. In: Yazıcı Y, Yazıcı H, eds. Behçet’s Syndrome. New York, NY: Springer; 2010:215-241.
7. Treatment. American Behçet’s Disease Association website. behcets.com. Accessed October 4, 2018.
8. Kural-Seyahi E, Fresko I, Seyahi N, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore). 2003;82(1):60-76.
9. Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol. 2013;9(2):79-89.
10. Alpsoy E. Behçet’s disease: a comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016;43(6):620-632.
11. Canpolat O, Yurtsever S. The quality of life in patients with Behçet’s disease. Asian Nurs Res (Korean Soc Nurs Sci). 2011;5(4):229-235.
12. Senusi A, Higgins S, Fortune F. The influence of oral health and psycho-social well-being on clinical outcomes in Behçet’s disease. Rheumatol Int. 2018;38(10):1873-1883.
13. Mumcu G, Inanc N, Ergun T, et al. Oral health related quality of life is affected by disease activity in Behçet’s disease. Oral Dis. 2006;12(2):145-151.
14. Ertam I, Kitapcioglu G, Aksu K, et al. Quality of life and its relation with disease severity in Behçet’s disease. Clin Exp Rheumatol. 2009;27(2 suppl 53):S18-S22.
15. Bang D, Hur W, Lee ES, Lee S. Prognosis and clinical relevance of recurrent oral ulceration in Behçet's disease. J Dermatol. 1995;22(12):926-929.
16. Sola-Morales O, Mehta S, Ronnebaum S, Patel D, Nazareth T, Padula A. A systematic literature review of healthcare burden in patients with Behçet’s disease. Presented at: the Annual European Congress of Rheumatology (EULAR 2019); 12-15 June 2019; Madrid, Spain.
17. Cho SB, Cho S, Bang D. New insights in the clinical understanding of Behçet’s disease. Yonsei Med J. 2012;53(1):35-42.
18. Galeone M, Colucci R, D'Erme AM, Moretti S, Lotti T. Potential infectious etiology of Behçet's disease. Patholog Res Int. 2012;2012:595380.
19. Nanke Y, Yago T, Kotake S. The role of Th17 cells in the pathogenesis of Behcet's disease. J Clin Med. 2017:6(7). pii: E74.
20. Lopalco G, Lucherini OM, Lopalco A, et al. Cytokine signatures in mucocutaneous and ocular Behçet's disease. Front Immunol. 2017;8:200.
21. Na SY, Park MJ, Park S, Lee ES. Up-regulation of Th17 and related cytokines in Behçet’s disease corresponding to disease activity. Clin Exp Rheumatol. 2013;31(3 suppl 77):32-40.
22. Chi W, Zhu X, Yang P, et al. Upregulated IL-23 and IL-17 in Behçet patients with active uveitis. Invest Ophthalmol Vis Sci. 2008;49(7):3058-3064.
23. Cantarini L, Pucino V, Vitale A, et al. Immunometabolic biomarkers of inflammation in Behçet’s disease: relationship with epidemiological profile, disease activity and therapeutic regimens. Clin Exp lmmunol. 2016;184(2):197-207.
24. Hatemi G, Seyahi E, Fresko I, Talarico R, Hamuryudan V. One year in review 2017: Behçet's syndrome. Clin Exp Rheumatol. 2017;35(suppl 108):3-15.
25. Direskeneli H, Saruhan-Direskeneli G. The role of heat shock proteins in Behçet’s disease. Clin Exp Rheumatol. 2003;21(4 suppl 30):S44-S48.
