View source for Behçet's disease

{{Distinguish|Becker disease}}
{{Infobox disease |
  Name           = Behçet disease |
  Image          =|
  Caption        = Bechet eye lesions|
  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)  ({{pron-en|bɛˈtʃɛt}}) is a form of [[vasculitis]]<ref name="urlGlossary, ">{{cite web |url=http://medicalcenter.osu.edu/patientcare/healthcare_services/multiple_sclerosis/ms_glossary/Pages/index.aspx |title=Glossary, |format= |work= |accessdate=2009-03-28}}</ref> that can lead to ulceration and other lesions. It can be interpreted as a chronic disturbance 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.

The [[etiology]] is not well-defined, but it is characterized by inflammation of the blood vessels. Although sometimes erroneously referred to as a "diagnosis of exclusion," the diagnosis can be reached by pathologic examination of the affected areas.<ref name="urlBehcet Disease: Overview - eMedicine Dermatology">{{cite web |url=http://emedicine.medscape.com/article/1122381-overview |title=Behcet Disease: Overview - eMedicine Dermatology |format= |work= |accessdate=2009-03-28}}</ref>

==History==
Behçet disease is [[eponym|named]] after [[Hulusi Behçet]] (1889–1948), the [[Turkish people|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" />

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 described "neuro-Behçet's disease",<ref>Ravi Malhotra (2004), "Saudi Arabia", ''Practical Neurology'' '''4''': 184-185.</ref> a [[Neurology|neurological]] involvement in Behçet's disease, considered one of the most devastating manifestations of the disease as investigated by an Egyptian researcher Sahar Saleem.<ref>S. Saleem (2005), [http://www.neurographics.org/4/2/1/4.shtml Neuro-Behçet's Disease: NBD], ''Neurographics'', Vol. 4, Issue 2, Article 1.</ref> The mechanism can be immune-mediated, or thrombotic.<ref name="pmid19161910">{{cite journal |author=Al-Araji A, Kidd DP |title=Neuro-Behçet's disease: epidemiology, clinical characteristics, and management |journal=Lancet Neurol |volume=8 |issue=2 |pages=192–204 |year=2009 |month=February |pmid=19161910 |doi=10.1016/S1474-4422(09)70015-8 |url=http://linkinghub.elsevier.com/retrieve/pii/S1474-4422(09)70015-8}}</ref> The term dates back to at least 1990.<ref name="pmid2188002">{{cite journal |author=Su SL, Way LJ, Lin RT, Peng MJ, Wu SC |title=Neuro-Behçet's disease: report of three cases with a review of the literature |journal=Gaoxiong Yi Xue Ke Xue Za Zhi |volume=6 |issue=3 |pages=155–62 |year=1990 |month=March |pmid=2188002 |doi= |url=}}</ref>

==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<ref name="pmid1970380">{{cite journal |author= |title=Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease |journal=Lancet |volume=335 |issue=8697 |pages=1078–80 |year=1990 |month=May |pmid=1970380 |doi= |url=}}</ref> (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===
According to the Internation Study Group for Behçet's disease,<ref name="pmid1970380">{{cite journal
|author=
|title=Criteria for diagnosis of Behçet's disease. International Study  Group for Behçet's Disease
|journal=Lancet
|volume=335
|issue=8697
|pages=1078–80
|year=1990
|month=May
|pmid=1970380
|doi=10.1016/0140-6736(90)92643-V
|last1=Internationalstudygroupforbehc
}}</ref> to be diagnosed with this condition a person 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 [[anus|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&nbsp;mm dia. 24-48 hrs or more after needle-prick).

===Practical clinical diagnosis===

[[image:Behcet.JPG|260px|thumb|right|Magnetic resonance venogram demonstrating occlusion of the left sigmoid and transverse sinuses.]]
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 Behçet 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. First degree relatives are affected in more than expected proportion of patients. 

It is rare in the United States, but is common in the Middle East and Asia, suggesting a cause endemic to the tropical areas.<ref>[http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html Behcet's syndrome (Medline Plus).]</ref>

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]].  However, one study has revealed a possible connection to food allergies, particularly to dairy products.<ref>Triolo ''et al.'' (2002). "Humoral and cell mediated immune response to cow's milk proteins in Behçet's disease." ''Ann Rheum Dis'' '''61'''(5):459-62. PMID 11959773.</ref>

==Treatment==
Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Corticosteroid therapy (1&nbsp;mg/kg/d oral prednisone) is indicated for severe disease manifestations<ref>CMDT (Current Medical Diagnosis & Treatment) 2007, Chapter 20, page 872</ref>. 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  | pmid=11498218 | doi=10.1016/S0140-6736(01)05497-6}}</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  | pmid=12379622 | pmc=1766720}}</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  | 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  | pmid=11939808 | doi=10.1001/archderm.138.4.467}}</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  | pmid=12642304 | doi=10.1136/bjo.87.4.423 | pmc=1771623}}</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  | pmid=12455182}}</ref> and [[Colchicine]] can 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  | pmid=11710724 | doi=10.1002/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H}}</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  | 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  | pmid=12568631 | doi=10.2165/00126839-200304010-00002}}</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  | pmid=12081158}}</ref>

[[Image:HLA-B*5101.png|thumb|left|[[HLA-B51]] is strongly associated with Behçet's disease<ref>{{Cite journal| volume = 100 | issue = 9 | pages = 1455–1458 | last = Ohno | first = S. | coauthors = M. Ohguchi, S. Hirose, H. Matsuda, A. Wakisaka, M. Aizawa | title = Close association of HLA-Bw51 with Behcet's disease | journal = Archives of Ophthalmology
| year = 1982| pmid = 6956266}}</ref>]]

==Pathophysiology==
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. A large number of serological studies show a linkage between the disease and [[HLA-B51]].<ref name="pmid18214795">{{cite journal |author=Durrani K, Papaliodis GN |title=The genetics of Adamantiades-Behcet's disease |journal=Semin Ophthalmol |volume=23 |issue=1 |pages=73–9 |year=2008 |pmid=18214795 |doi=10.1080/08820530701745264 }}</ref> HLA-B51 is more frequently found from the Middle East to South Eastern Siberia, but the incidence of B51 in some studies was 3 fold higher than the normal population. However, B51 tends not to be found in disease when a certain [[SUMO4]] gene variant is involved,<ref name="pmid18657476">{{cite journal |author=Hou S, Yang P, Du L, ''et al.'' |title=SUMO4 gene polymorphisms in Chinese Han patients with Behcet's disease |journal=Clin. Immunol. |volume= 129|issue= 1|pages= 170|year=2008 |month=July |pmid=18657476 |doi=10.1016/j.clim.2008.06.006 }}</ref> and symptoms appear to be milder when [[HLA-B27]] is present.<ref name="pmid17923546">{{cite journal |author=Ahn JK, Park YG |title=Human leukocyte antigen B27 and B51 double-positive Behçet uveitis |journal=Arch. Ophthalmol. |volume=125 |issue=10 |pages=1375–80 |year=2007 |month=October |pmid=17923546 |doi=10.1001/archopht.125.10.1375 }}</ref>  At the current time, a similar infectious origin has not yet been confirmed that leads to Behçet's disease, but certain strains of ''[[Streptococcus sanguinis]]'' has been found to have a homologous antigenicity.<ref name="pmid16514412">{{cite journal |author=Yanagihori H, Oyama N, Nakamura K, Mizuki N, Oguma K, Kaneko F |title=Role of IL-12B promoter polymorphism in Adamantiades-Behcet's disease susceptibility: An involvement of Th1 immunoreactivity against Streptococcus Sanguinis antigen |journal=J. Invest. Dermatol. |volume=126 |issue=7 |pages=1534–40 |year=2006 |month=July |pmid=16514412 |doi=10.1038/sj.jid.5700203 }}</ref>

==Epidemiology==
An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 1 case for every 100,000 people.<ref>[http://www.nhs.uk/Conditions/Behcets-disease/Pages/introduction.aspx?url=Pages/what-is-it.aspx Behcet's disease.]</ref> Globally, males are affected more frequently than females.<ref name="pmid16476027">{{cite journal |author=Escudier M, Bagan J, Scully C |title=Number VII Behçet's disease (Adamantiades syndrome) |journal=Oral Dis |volume=12 |issue=2 |pages=78–84 |year=2006 |month=March |pmid=16476027 |doi=10.1111/j.1601-0825.2005.01144.x |url=}}</ref> In the United States, more females are affected than males.{{Citation needed|date=August 2008}}
{{clr}}

== Pronunciation ==
Because [[Hulusi Behçet]] was Turkish, the correct pronunciation is with a hard "ch", as in "choice", with "e" (both first and second e letters) as in "end" and with the terminal "t" sounded: "Beh-chet". 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 "e" incorrectly like "i" (as in see), with the "t" incorrectly silenced: "Beshay".

== See also ==
* [[Behcet's Disease of the Optic Nerve]]
* [[List of cutaneous conditions]]

==References==
{{Reflist|2}}

==External links==
* {{DMOZ|Health/Conditions_and_Diseases/Musculoskeletal_Disorders/Connective_Tissue/Behcet's_Syndrome/}}

{{Systemic connective tissue disorders}}

{{DEFAULTSORT:Behcet's disease}}
[[Category:Autoimmune diseases]]
[[Category:Conditions of the mucous membranes]]
[[Category:Words of Turkish origin]]
[[Category:Inflammations]]
[[Category:Systemic connective tissue disorders]]

[[ar:مرض بهجت]]
[[ca:Malaltia de Behçet]]
[[de:Morbus Adamantiades-Behçet]]
[[es:Síndrome de Behcet]]
[[fr:Maladie de Behçet]]
[[it:Sindrome di Behçet]]
[[he:מחלת בכצ'ט]]
[[nl:Ziekte van Behçet]]
[[ja:ベーチェット病]]
[[pl:Choroba Behçeta]]
[[pt:Doença de Behçet]]
[[ru:Болезнь Бехчета]]
[[sr:Бехчетов синдром]]
[[fi:Behçetin tauti]]
[[tr:Behçet hastalığı]]
[[uk:Синдром Бехчета]]
[[zh:貝賽特氏症]]
[[Category:2Fix]]