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+#REDIRECT [[Adamantiades-Behçet's-disease]]
-{{Infobox_Disease |
-  Name           = Behçet disease |
-  Image          = |
-  Caption        = |
-  DiseasesDB     = 1285 |
-  ICD10          = {{ICD10|M|35|2|m|30}} |
-  ICD9           = {{ICD9|279.4}} |
-  ICDO           = |
-  OMIM           = 109650 |
-  MedlinePlus    = |
-  eMedicineSubj  = med |
-  eMedicineTopic = 218 |
-  eMedicine_mult = {{eMedicine2|ped|219}} {{eMedicine2|derm|49}} {{eMedicine2|oph|425}} |
-  MeshID         = D001528 |
-}}
-'''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 [[infection]]s through controlled [[inflammation]], becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects [[blood vessel]]s, 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 [[eponym|named]] after [[Hulusi Behçet]] (1889-1948), the [[Turkey|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 name="WhoNamedIt">{{WhoNamedIt|synd|1863}}</ref><ref>H. Behçet. Über rezidivierende, aphtöse, durch ein Virus verursachte Geschwüre am Mund, am Auge und an den Genitalien.
-Dermatologische Wochenschrift, Hamburg, 1937, 105(36): 1152-1163.</ref>  The name (''Morbus Behçet'') was formally adopted at the International Congress of Dermatology in [[Geneva]] in September 1947.
-Symptoms of this disease may have been described by [[Hippocrates]] in the [[5th century BC]], in his 3rd Epidemion-book.<ref>Johns Hopkins Vasculitis Center (2004). [http://vasculitis.med.jhu.edu/typesof/behcets.html Johns Hopkins Vasculitis Center Discusses Behcets Disease]. Retrieved September 9, 2005.</ref> Its first modern formal description was published in 1922.<ref name="WhoNamedIt"> </ref>
-Some sources use the term "Adamantiades’ syndrome" or "Adamandiades-Behçet syndrome", for the work done by [[Benediktos Adamantiades]].<ref>B. Adamandiades. Sur un cas d'iritis à hypopyon récidivant. Annales d'oculistique, Paris, 1931, 168: 271-278.</ref> However, the current [[World Health Organization]]/[[ICD-10]] standard is "Behçet's disease".
-In 1991, [[Saudi Arabia]]n medical researchers discovered "neuro-Behcet's disease",<ref>Ravi Malhotra (2004), "Saudi Arabia", ''Practical Neurology'' '''4''': 184-185.</ref> a [[Neurology|neurological]] involvement in Behcet's disease, considered one of the most devastating manifestations of the disease.<ref>S. Saleem (2005), [http://www.neurographics.org/4/2/1/4.shtml Neuro-Behcet's Disease: NBD], ''Neurographics'', Vol. 4, Issue 2, Article 1.</ref>
-==Pathology==
-The symptoms of Behçet 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, sore [[genital]]s 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]], [[irritable bowel syndrome|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 [[remission]]s 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 [[testicle]]s or [[epididymitis]] in men),
-*[[skin]] [[lesion]]s (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 [[wikt:personality|personality]], [[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 Behçet 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 [[Ageing|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.<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>
-Interferon alfa-2a may also be an effective alternative treatment, particularly for the 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 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]] can be useful for treating some genital ulcers, erythema nodosum, and arthritis in women, and arthritis in men.<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>
-[[Thalidomide]] has also 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>
-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]].  Ankylosing spondylitis is not due to an 'oveactive' immune system; instead 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. At the current time, a similar infectious origin has not yet been confirmed that leads to Behçet disease, but certain strains of ''Streptococcus sanguis'' has been found to have a homologous antigenicity.
-==Epidemiology==
-Behçet disease is considered more prevalent in the areas surrounding the [[Silk Road|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==
-{{Reflist|2}}
-==External links==
-*[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
-* [http://www.hulusibehcet.net/manifest.htm Signs & Symptoms of Behçet's disease]  (with pictures)
-* [http://www.behcets.com/home.ivnu American Behçet's Disease Association]
-* [http://www.behcets-society.fsnet.co.uk/index.html Behçet's Syndrome Society] (UK)
-* [http://www.behcet.ws/ International Society for Behçet's Disease]
-* {{DermNet|systemic/behcet}}
-{{Diseases of the musculoskeletal system and connective tissue}}
-[[Category:Autoimmune diseases]]
-[[Category:Dermatology]]
-[[de:Morbus Adamantiades-Behçet]]
-[[es:Síndrome de Behcet]]
-[[fr:Maladie de Behçet]]
-[[he:תסמונת בכצ'ט]]
-[[it:Sindrome di Behçet]]
-[[nl:Ziekte van Behçet]]
-[[ja:ベーチェット病]]
-[[pl:Choroba Behçeta]]
-[[pt:Doença de Behçet]]
-[[tr:Behçet hastalığı]]
-[[zh:貝賽特氏症]]