(Redirected from Behçet\'s syndrome)
'Behçet disease' (Behçet's syndrome, ''Morbus Behçet'', silk road disease) is a chronic condition due to disturbances in the body’s
immune system. This system, which normally protects the body against
infections through controlled
inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects
blood vessels, usually the small ones. As a result,
symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a
blood supply.
History
Behçet disease is
named after
Hulusi Behçet (1889-1948), the
Turkish dermatologist and
scientist who first recognized the syndrome in one of his patients in 1924 and reported his research on the disease in ''Journal of Skin and Veneral Diseases'' in 1936.
[1] The name (''Morbus Behçet'') was formally adopted at the International Congress of Dermatology in
Geneva in September 1947.
The disease was probably first described by
Hippocrates in the
5th century BC, in his 3rd Epidemion-book.
[2]
Pathology
The symptoms of Behçet disease are believed to be caused by an over-active
immune system which, without any apparent
infections, produces recurrent outbreaks of inflammation in small blood vessels. Common symptoms include
mouth ulcers, sore
genitals and
eye inflammation, and
arthritis in older patients, mostly painful but not life-threatening conditions. However, some patients may be unable to work because of the pain and the impaired vision and mobility. In some severe cases, uncontrolled inflammation may lead to
blindness,
intestinal complications,
stroke, and even
meningitis, which can be fatal.
This disease usually first strikes patients in their 20s and 30s. It then becomes a fluctuating lifelong disorder with a series of
remissions and exacerbations which can be from days to months. Complete remission is rare.
Diagnosis
There is no specific pathological test for Behçet disease at present. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks. Other causes for these symptoms have to be ruled out before making the diagnosis. The symptoms do not have to occur together, but can have happened at any time.
There are three levels of certainty for diagnosis:
#International Study Group diagnostic guidelines (very strict for research purposes)
#Practical clinical diagnosis (generally agreed pattern but not as strict)
#'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)
International Study Group diagnostic guidelines
Must have
★ oral (
aphthous) ulcers (any shape, size or number at least 3 times in any 12 months),
along with 2 out of the next 4 "
hallmark" symptoms:
★ genital ulcers (including
anal ulcers and spots in the genital region and swollen
testicles or
epididymitis in men),
★
skin lesions (papulo-pustules,
folliculitis,
erythema nodosum,
acne in post-adolescents not on corticosteroids),
★
eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous),
★ pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick).
Practical clinical diagnosis
Must have
★
mouth ulcers,
along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:
★
arthritis/
arthralgia,
★
nervous system symptoms,
★
stomach and/or
bowel inflammation,
★ deep
vein thrombosis,
★ superficial thrombophlebitis,
★ cardio-vascular problems of inflammatory origin,
★ inflammatory problems in
chest and
lungs,
★ problems with hearing and/or balance,
★ extreme exhaustion,
★ changes of ,
psychoses,
★ any other members of the family with a diagnosis of Behcet disease.
'Suspected' or 'Possible' diagnosis
Usually given when someone does not have mouth ulcers or has mouth ulcers but does not have 1 of the 4 hallmark symptoms but has other symptoms and signs of inflammation and other causes for these have been ruled out.
Causes
No one knows why the immune system starts to behave this way in Behcet disease. It is not because of any known infections, it is not
hereditary, it does not have to do with
ethnic origin,
gender, life-style, or
age, where someone has lived or where they have been on holiday. It is not associated with
cancer, and links with tissue-types (which are under investigation) are not certain. It does not follow the usual pattern for
autoimmune diseases.
Treatment
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as
infliximab has shown promise in treating the uveitis associated with the disease.
[3][4] Another Anti-TNF agent,
Etanercept, may be useful in patients with mainly skin and mucosal symptoms.
[5]
Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers
[6] as well as ocular lesions.
[7] Azathioprine, when used in combination with interferon alfa-2b also shows promise,
[8] and
Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis in women, and arthritis in men.
[9]
Thalidomide has also been used due to its immune-modifying effect.
[10] Dapsone and
rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions.
[11][12]
A different orientation could be explored in Behçet Disease, especially with genetic linkage to HLA-B51 antigen, just like the prevalence of HLA-B27 in Ankylosing Spondylitis, a very similar condition. AS is not due to an 'oveactive' immune system, but it is a true autoimmune disease caused by molecular mimicry of the Osp (outer surface protein) with the Klebsiella pneumoniae germ (2 enzymes produced by this normally non-virulent pathogen), which is always present as a sub-clinical infection, typically at the ileocecal junction. The combination of antibiotics targeted to this specific germ, and dietary controls (elimination or severe restriction of all starches) could therefore potentially provide the most effective treatments, but such treatments have not yet been proven or generally approved.
Epidemiology
Behçet disease is considered more prevalent in the areas surrounding the
old silk trading routes in the
Middle East and in
Central Asia. Thus, it is sometimes known as ''Silk Road Disease''. However, this disease is not restricted to people from these regions.
An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 2 cases for every 100,000 people.
Globally, males are affected more frequently than females. In the United States, more females are affected than males.
Pronunciation note
Because it contains a
cedilla, "Behçet" is frequently wrongly assumed to be French in origin and pronounced with a sibilant "s" sound (as in "satsuma") or soft "ch" (as in "shoe"), with the "t" incorrectly silenced: "Beshay". Because
Hulusi Behçet was Turkish, the correct pronunciation is with a hard "ch", as in "choice", and with the terminal "t" sounded: "Beh-chet".
References
1.
2. Johns Hopkins Vasculitis Center (2004). Johns Hopkins Vasculitis Center Discusses Behcets Disease. Retrieved September 9, 2005.
3. Effect of infliximab on sight-threatening panuveitis in Behcet's disease, Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN, , , Lancet, 2001
4. Behcet's disease: a new target for anti-tumor necrosis factor treatment, Sfikakis PP, , , Ann Rheum Dis, 2002
5. Short-term trial of etanercept in Behcet's disease: a double blind, placebo controlled study, Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H, , , J Rheumatol, 2005
6. Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study, Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, Basaran E, , , Arch Dermatol, 2002
7. Human recombinant interferon alfa-2a for the treatment of Behcet's disease with sight threatening posterior or panuveitis, Kotter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Gunaydin I, Grimbacher B, Blaschke S, Meyer-Riemann W, Peter HH, Stubiger N, , , Br J Ophthalmol, 2003
8. Interferon alfa combined with azathioprine for the uveitis of Behcet's disease: an open study, Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H, , , Isr Med Assoc J, 2002
9. A double-blind trial of colchicine in Behcet's syndrome, Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, Senocak M, Yazici H, , , Arthritis Rheum, 2001
10. Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial, Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, Zwingenberger K, Yazici H, , , Ann Intern Med, 1998
11. Efficacy of rebamipide as adjunctive therapy in the treatment of recurrent oral aphthous ulcers in patients with Behcet's disease: a randomised, double-blind, placebo-controlled study, Matsuda T, Ohno S, Hirohata S, Miyanaga Y, Ujihara H, Inaba G, Nakamura S, Tanaka S, Kogure M, Mizushima Y, , , Drugs R D, 2003
12. Dapsone in Behcet's disease: a double-blind, placebo-controlled, cross-over study, Sharquie KE, Najim RA, Abu-Raghif AR, , , J Dermatol, 2002
External links
★
Behçet's Disease Resource Guide from the National Eye Institute (NEI).
★
Behcet disease - ''MedLink Neurology'' Clinical Summary
★
Signs & Symptoms of Behçet's disease (with pictures)
★
American Behçet's Disease Association
★
Behçet's Syndrome Society (UK)
★
International Society for Behçet's Disease
★
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