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About Behçet's Disease

Manifestations of Behçet's Disease

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 vein of all sizes. The most common manifestation is mucocutaneous lesions accompanied by lesions in other organ systems.1-4

Behçet’s affects multiple organ systems and presents to multiple different medical specialties1,2,5,6

OCULAR DISEASE

50%

+

OCULAR DISEASE

50%

Including: Anterior uveitis, relapsing hypopyon, vitritis, retinal infiltrates, retinal vasculitis, retinal vascular occlusion.

Presenting specialist: Ophthalmologist.

View an image of ocular disease here.

VASCULAR

16%

+

VASCULAR

16%

Including: Ischemic heart disease; cardiac, pulmonary, and peripheral vessel aneurysms; deep venous occlusions.

Presenting specialists: Cardiologist, pulmonologist, vascular surgeon.

GASTROINTESTINAL

0%-25%

+

GASTROINTESTINAL

0%-25%

Including: Aphthous ulcers in any part of the GI tract.

Presenting healthcare professionals: Primary care physician, gastroenterologist.

GENITAL ULCERS

80%

+

GENITAL ULCERS

80%

Including: Scrotal lesions, vulval lesions, epididymitis.

Presenting specialists: Urologist, gynecologist.

View images of genital ulcers here.

NEUROLOGICAL

5%-25%

+

NEUROLOGICAL

5%-25%

Including: Parenchymal disease, cerebral sinus thrombosis.

Presenting specialist: Neurologist.

ORAL ULCERS

98%

+

ORAL ULCERS

98%

Including: Aphthous ulcers in the mouth and/or throat.

Presenting healthcare professionals: Primary care physician, dentist, oral surgeon.

View images of oral ulcers here.

SKIN LESIONS

80%

+

SKIN LESIONS

80%

Including: Folliculitis, erythema nodosum, pathergy response.

Presenting specialist: Dermatologist.

View images of skin lesions here.

JOINTS

45%

+

JOINTS

45%

Including: Non-obliterative arthritis, usually in large joints.

Presenting specialist: Rheumatologist.

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 primary care physician. 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

Images of Behçet’s disease manifestations

Frequency of Behçet’s Disease manifestations first observed by a healthcare professional8,a

Oral Ulcers

100%

Genital Ulcers

95%

Papulopustular Lesions

83%

Erythema Nodosum

62%

Eye Disease

47%

Arthritis

39%

Vascular Disease

21%

Neurologic Disease

3%

Patients (%)

aStudy of 428 patients whose first clinical visits were between July 1977 and December 1983 (inclusive) in the multidisciplinary Behçet's Disease outpatient clinic at Cerrahpaşa Medical Faculty of the University of Istanbul, Turkey.8

The manifestations of Behçet's Disease 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

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

Cause of Behçet's 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.3 The exact mechanism of the inflammatory changes in Behçet's Disease remains unknown.4

Behçet’s: An uncontrolled autoimmune cascade in vascular tissue3,10,16-23

Environmental Triggers

  • Viral or bacterial agents such as Streptococcus sanguis, herpes simplex virus, Epstein-Barr virus and cytomegalovirus
  • Heat shock proteins
  • Pollution

Genetic Predisposition

  • Family history
  • HLA-B51
  • IL-10
  • IL23R/IL12RB2

Immune Response Activation

Increased Production Of Proinflammatory Cytokines

IL-2, IL-6, IL-12, IL-17, IL-23, IL-33, TNF-alpha, IFN-gamma

Inflammation

HLA, human leukocyte antigen; IFN, interferon; IL, interleukin; TNF, tumor necrosis factor.

Management of Behçet’s Disease

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 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.

References:

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., C. Demirkesen et al. History and diagnosis. In: Yazıcı Y, Yazıcı H, eds. Behçet’s Syndrome. New York, NY: Springer; 2010:7-34. 

3. Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P. Behçet’s disease physiopathology: a contemporary review. Autoimmun Highlights. 2016:7(1):4. 

4. Leonardo NM, McNeil J. Behcet'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. History and diagnosis. In: Yazıcı Y, Yazıcı H, eds. Behçet’s Syndrome. New York, NY: Springer; 2010:215-241.

7. Treatment. American Behçet’s Disease Association website. http://www.behcets.com/site/c.8olJJRPsGclSF/b.9145379/k.FED2/Treatment.htm. 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;43:620-632. 

11. Canpolat Ö, 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;12:145-151. 

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. Cho SB, Cho S, Bang D. New insights in the clinical understanding of Behçet’s disease. Yonsei Med J. 2012:53(1):35-42. 

17. Galeone M, Colucci R, D'Erme AM, Moretti S, Lotti T. Potential infectious etiology of Behçet's disease. Pathology Research Int. 2012:2012:595380. 

18. 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. 

19. Lopalco G, Lucherini OM, Lopalco A, et al. Cytokine signatures in mucocutaneous and ocular Behçet's Disease. Front Immunol. 2017;8:200. 

20. 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. 

21. 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. 

22. Cantarini L, Pucino V, Vitale A, et al. lmmunometabolic biomarkers of inflammation in Behçet’s disease: relationship with epidemiological profile, disease activity and therapeutic regimens. Clin Exp lmmunol. 2016;184:197-207. 

23. 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):S3-S15. 

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