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| {{Infobox_Disease | | | {{Behçet's disease}} |
| Name = Behçet disease |
| | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| Image = |
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| Caption = |
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| DiseasesDB = 1285 |
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| ICD10 = {{ICD10|M|35|2|m|30}} |
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| ICD9 = {{ICD9|279.4}} |
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| ICDO = |
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| OMIM = 109650 |
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| MedlinePlus = |
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| eMedicineSubj = med |
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| eMedicineTopic = 218 |
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| eMedicine_mult = {{eMedicine2|ped|219}} {{eMedicine2|derm|49}} {{eMedicine2|oph|425}} |
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| MeshID = D001528 |
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| }}
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| {{Behcet's disease}} | | {{CMG}}; {{AE}} {{HQ}} |
| '''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
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| {{CMG}} | |
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| {{CZ}} | | {{SK}} Behcet disease; Behçet syndrome; oculobuccogenital syndrome; Behçet's Syndrome |
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| ===[[The Heart in Behçet's disease|For the heart in Behçet's disease click here]]=== | | ===[[The Heart in Behçet's disease|For the heart in Behçet's disease click here]]=== |
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| ==Overview== | | ==[[Behçet's disease overview|Overview]]== |
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| '''Behçet's disease''' (Behçet's syndrome, ''Morbus Behçet'', silk road disease) is a chronic inflammatory condition caused by disturbances in the [[immune system]]. The immune system normally protects the body against [[infection]]s through a controlled [[inflammatory reaction]] against the pathogen.In Behcet's disease, the immune response becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects [[blood vessel]]s, in particular the smaller vessels. As a result, [[symptoms]] appear wherever the exaggerated inflammation response is produced, and can occur anywhere on the body where there is a [[blood]] supply.
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| ==History== | | ==[[Behçet's disease historical perspective|Historical Perspective]]== |
| Behçet's 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 Venereal Diseases'' in 1936.<ref>{{WhoNamedIt|synd|1863}}</ref> The name (''Morbus Behçet'') was formally adopted at the International Congress of Dermatology in Geneva in September 1947. | |
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| A group of similar symptoms were first described by [[Hippocrates]] in 5th century BC as part of his 3rd Epidemion book.<ref>Johns Hopkins Vasculitis Center (2004). [http://vasculitis.med.jhu.edu/typesof/behcets.html Johns Hopkins Vasculitis Center Discusses Behçets Disease]. Retrieved September 9, 2005.</ref>
| | ==[[Behçet's disease classification|Classification]]== |
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| ==Pathology== | | ==[[Behçet's disease pathophysiology|Pathophysiology]]== |
| The symptoms of Behçet's disease are believed to be caused by an over-active [[immune system]] which, without any apparent [[infection]]s, produces recurrent outbreaks of inflammation in small blood vessels. Common symptoms include [[mouth ulcer]]s,[[genital ulcer]]s, [[eye]] inflammation, and [[arthritis]] in older patients. The symptoms are mostly painful but not life threatening. Some patients however experience significant impairment due to pain, impaired vision and reduced mobility, and may be disabled and unable to work due to this condition. In some severe cases, uncontrolled inflammation may lead to [[blindness]], [[irritable bowel syndrome|intestinal complications]], [[stroke]], and even [[meningitis]], which can be fatal.
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| Behcet's disease usually first affects patients when they are in their 20s and 30s. It then becomes a fluctuating lifelong disorder with a series of [[remission]]s and exacerbations which can last from days to months. Complete remission is rare.
| | ==[[Behçet's disease causes|Causes]]== |
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| ==Diagnosis== | | ==[[Behçet's disease differential diagnosis|Differentiating Behçet's disease from Other Diseases]]== |
| There is no specific test to confirm the presence of Behçet's disease currently. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patients symptoms (outlined below). Behcet's disease is a diagnosis of exclusion, and other chronic inflammatory diseases should be evaluated for. The various inflammatory symptoms do not necessarily occur together, and will vary in severity.
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| There are three levels of certainty for diagnosis:
| | ==[[Behçet's disease epidemiology and demographics|Epidemiology and Demographics]]== |
| #International Study Group diagnostic guidelines (very strict for research purposes)
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| #Practical clinical diagnosis (generally agreed pattern but not as strict)
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| #'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)
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| ===International Study Group diagnostic guidelines=== | | ==[[Behçet's disease risk factors|Risk Factors]]== |
| Must have
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| *oral ([[aphthous]]) ulcers (any shape, size or number at least 3 times in any 12 months),
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| along with 2 out of the next 4 "hallmark" symptoms:
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| *genital ulcers (including [[anal]] ulcers and spots in the genital region and swollen [[testicle]]s or [[epididymitis]] in men),
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| *[[skin]] [[lesion]]s (papulo-pustules, [[folliculitis]], [[erythema nodosum]], [[acne]] in post-adolescents not on corticosteroids),
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| *[[eye]] inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous),
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| *pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick).
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| ===Practical clinical diagnosis=== | | ==[[Behçet's disease natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| Must have
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| *[[mouth]] ulcers,
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| along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:
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| *[[arthritis]]/[[arthralgia]],
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| *[[nervous system]] symptoms,
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| *[[stomach]] and/or [[bowel]] inflammation,
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| *deep [[vein]] [[thrombosis]],
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| *superficial thrombophlebitis,
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| *cardio-vascular problems of inflammatory origin,
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| *inflammatory problems in [[chest]] and [[lungs]],
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| *problems with hearing and/or balance,
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| *extreme exhaustion,
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| *changes of [[wikt:personality|personality]], [[psychoses]],
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| *any other members of the family with a diagnosis of Behcet disease.
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| ==='Suspected' or 'Possible' diagnosis=== | | ==Diagnosis== |
| Usually assigned 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.
| | [[Behçet's disease diagnostic study of choice|Diagnostic Study of Choice]] | [[Behçet's disease history and symptoms|History and Symptoms]] | [[Behçet's disease physical examination|Physical Examination]] | [[Behçet's disease laboratory findings|Laboratory Findings]] | [[Behçet's disease electrocardiogram|Electrocardiogram]] | [[Behçet's disease x ray|X Ray]] | [[Behçet's disease CT|CT]] | [[Behçet's disease MRI|MRI]] | [[Behçet's disease echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Behçet's disease other imaging findings|Other Imaging Findings]] | [[Behçet's disease other diagnostic studies|Other Diagnostic Studies]] |
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| ==Causes==
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| The reason why the immune system behaves in this manner in Behcet's disease is unknown. It does not occur in association with any known infections, it is not [[hereditary]], it is not associated with ethnic origin, [[gender]], life-style, or [[Aging|age]], location, or travel. It is not associated with [[cancer]], and links with particular tissue-types (which are under investigation) are not certain. It does not follow the usual pattern for other [[autoimmune diseases]].
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| ==Treatment== | | ==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.<ref>{{cite journal | author=Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN | title=Effect of infliximab on sight-threatening panuveitis in Behcet's disease | journal=Lancet | year=2001 | pages=295-6 | volume=358 | issue=9278 | id=PMID 11498218}}</ref><ref>{{cite journal | author=Sfikakis PP | title=Behcet's disease: a new target for anti-tumor necrosis factor treatment | journal=Ann Rheum Dis | year=2002 | pages=ii51-3 | volume=61 Suppl 2 | id=PMID 12379622}}</ref> Another Anti-TNF agent, [[etanercept]], may be useful in patients with mainly skin and mucosal symptoms.<ref>{{cite journal | author=Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H | title=Short-term trial of etanercept in Behcet's disease: a double blind, placebo controlled study | journal=J Rheumatol | year=2005 | pages=98-105 | volume=32 | issue=1 | id=PMID 15630733}}</ref>
| | [[Behçet's disease medical therapy|Medical Therapy]] | [[Behçet's disease primary prevention|Primary Prevention]]| [[Behçet's disease secondary prevention|Secondary Prevention]] | [[Behçet's disease surgery|Surgery]] | [[Behçet's disease cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Behçet's disease future or investigational therapies|Future or Investigational Therapies]] |
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| Interferon alfa-2a may also be an effective alternative treatment, particularly for patients with genital and oral ulcers<ref>{{cite journal | author=Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, Basaran E | title=Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study | journal=Arch Dermatol | year=2002 | pages=467-71 | volume=138 | issue=4 | id=PMID 11939808}}</ref> as well as with ocular lesions.<ref>{{cite journal | author=Kotter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Gunaydin I, Grimbacher B, Blaschke S, Meyer-Riemann W, Peter HH, Stubiger N | title=Human recombinant interferon alfa-2a for the treatment of Behcet's disease with sight threatening posterior or panuveitis | journal=Br J Ophthalmol | year=2003 | pages=423-31 | volume=87 | issue=4 | id=PMID 12642304}}</ref> [[Azathioprine]], when used in combination with interferon alfa-2b also shows promise,<ref>{{cite journal | author=Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H | title=Interferon alfa combined with azathioprine for the uveitis of Behcet's disease: an open study | journal=Isr Med Assoc J | year=2002 | pages=928-30 | volume=4 | issue=11 Suppl | id=PMID 12455182}}</ref> and [[Colchicine]] may also be useful for treating some genital ulcers, erythema nodosum, and arthritis.<ref>{{cite journal | author=Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, Senocak M, Yazici H | title=A double-blind trial of colchicine in Behcet's syndrome | journal=Arthritis Rheum | year=2001 | pages=2686-92 | volume=44 | issue=11 | id=PMID 11710724}}</ref>
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| [[Thalidomide]] has been used due to its immune-modifying effect.<ref>{{cite journal | author=Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, Zwingenberger K, Yazici H | title=Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial | journal=Ann Intern Med | year=1998 | pages=443-50 | volume=128 | issue=6 | id=PMID 9499327}}</ref> [[Dapsone]] and [[rebamipide]] have been shown, in small studies, to have beneficial results for mucocutaneous lesions.<ref>{{cite journal | author=Matsuda T, Ohno S, Hirohata S, Miyanaga Y, Ujihara H, Inaba G, Nakamura S, Tanaka S, Kogure M, Mizushima Y | title=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 | journal=Drugs R D | year=2003 | pages=19-28 | volume=4 | issue=1 | id=PMID 12568631}}</ref><ref>{{cite journal | author=Sharquie KE, Najim RA, Abu-Raghif AR | title=Dapsone in Behcet's disease: a double-blind, placebo-controlled, cross-over study | journal=J Dermatol | year=2002 | pages=267-79 | volume=29 | issue=5 | id=PMID 12081158}}</ref>
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| A different origin could be explored in Behçet's disease particularly in regards to genetic linkage to HLA-B51 antigen, similar to the prevalence of HLA-B27 in Ankylosing Spondylitis which is a similar condition. Ankylosing spondylitis is not due to an 'overactive' immune system, but 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.
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| ==Epidemiology==
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| 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.
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| 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.
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| Globally, males are affected more frequently than females. In the United States, more females are affected than males.
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| == Pronunciation note ==
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| '''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".'''
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| ==References==
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| {{Reflist|2}}
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| | ==Case Studies== |
| | :[[Behçet's disease case study one|Case #1]] |
| ==External links== | | ==External links== |
| *[http://www.nei.nih.gov/health/behcets/index.asp Behçet's Disease] Resource Guide from the National Eye Institute (NEI). | | *[http://www.nei.nih.gov/health/behcets/index.asp Behçet's Disease] Resource Guide from the National Eye Institute (NEI). |
| * [http://www.medlink.com/PublicCIP.ASP?access=public&UID=MLT000P2&code= Behcet disease] - ''MedLink Neurology'' Clinical Summary
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| * [http://www.hulusibehcet.net/manifest.htm Signs & Symptoms of Behçet's disease] (with pictures)
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| * [http://www.behcets.com/home.ivnu American Behçet's Disease Association]
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| * [http://www.behcets-society.fsnet.co.uk/index.html Behçet's Syndrome Society] (UK)
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| * [http://www.behcet.ws/ International Society for Behçet's Disease]
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| * {{DermNet|systemic/behcet}}
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| {{Diseases of the musculoskeletal system and connective tissue}} | | {{Diseases of the musculoskeletal system and connective tissue}} |
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| [[Category:Autoimmune diseases]]
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| [[Category:Dermatology]]
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| [[Category:Rheumatology]]
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| [[de:Morbus Behçet]] | | [[de:Morbus Behçet]] |
| [[es:Síndrome de Behcet]] | | [[es:Síndrome de Behcet]] |
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