Behçet's disease

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Not to be confused with Becker disease.
Behçet disease
Classification and external resources
ICD-10 M35.2
ICD-9 279.4
OMIM 109650
DiseasesDB 1285
eMedicine med/218 ped/219 derm/49 oph/425
MeSH D001528

Behçet disease (Behçet's syndrome, Morbus Behçet, silk road disease) (pronounced /bɛˈtʃɛt/) is a form of vasculitis[1] 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 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.

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.[2]

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 Venereal Diseases in 1936.[3][4] 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.[5] Its first modern formal description was published in 1922.[3]

Some sources use the term "Adamantiades’ syndrome" or "Adamandiades-Behçet syndrome", for the work done by Benediktos Adamantiades.[6] However, the current World Health Organization/ICD-10 standard is "Behçet's disease".

In 1991, Saudi Arabian medical researchers described "neuro-Behçet's disease",[7] a neurological involvement in Behçet's disease, considered one of the most devastating manifestations of the disease as investigated by an Egyptian researcher Sahar Saleem.[8] The mechanism can be immune-mediated, or thrombotic.[9] The term dates back to at least 1990.[10]

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:

  1. International Study Group diagnostic guidelines[11] (very strict for research purposes)
  2. Practical clinical diagnosis (generally agreed pattern but not as strict)
  3. 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)

International Study Group diagnostic guidelines

According to the Internation Study Group for Behçet's disease,[11] 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:

Practical clinical diagnosis

File:Behcet.JPG
Magnetic resonance venogram demonstrating occlusion of the left sigmoid and transverse sinuses.

Must have

along with 1 of the 4 hallmark symptoms above and with 2 of the symptoms below:

'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.[12]

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.[13]

Treatment

Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Corticosteroid therapy (1 mg/kg/d oral prednisone) is indicated for severe disease manifestations[14]. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease.[15][16] Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms.[17]

Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers[18] as well as ocular lesions.[19] Azathioprine, when used in combination with interferon alfa-2b also shows promise,[20] and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis.[21]

Thalidomide has also been used due to its immune-modifying effect.[22] Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions.[23][24]

File:HLA-B*5101.png
HLA-B51 is strongly associated with Behçet's disease[25]

Pathophysiology

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. A large number of serological studies show a linkage between the disease and HLA-B51.[26] 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,[27] and symptoms appear to be milder when HLA-B27 is present.[28] 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.[29]

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.[30] Globally, males are affected more frequently than females.[31] In the United States, more females are affected than males.[citation needed]

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

References

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External links

General
Other hypersensitivity/autoimmune
Other

M: MUS, DF+DRCT

anat (h/n, u, t/d, a/p, l)/phys/hist

noco(m, s, c)/cong(d)/tumr, sysi/epon, injr

proc, drug (M1A/3)

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
  1. ↑ "Glossary,". Retrieved 2009-03-28. 
  2. ↑ "Behcet Disease: Overview - eMedicine Dermatology". Retrieved 2009-03-28. 
  3. ↑ 3.0 3.1 synd/1863 at Who Named It?
  4. ↑ 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.
  5. ↑ Johns Hopkins Vasculitis Center (2004). Johns Hopkins Vasculitis Center Discusses Behcets Disease. Retrieved September 9, 2005.
  6. ↑ B. Adamandiades. Sur un cas d'iritis à hypopyon récidivant. Annales d'oculistique, Paris, 1931, 168: 271-278.
  7. ↑ Ravi Malhotra (2004), "Saudi Arabia", Practical Neurology 4: 184-185.
  8. ↑ S. Saleem (2005), Neuro-Behçet's Disease: NBD, Neurographics, Vol. 4, Issue 2, Article 1.
  9. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  10. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  11. ↑ 11.0 11.1 Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  12. ↑ Behcet's syndrome (Medline Plus).
  13. ↑ 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.
  14. ↑ CMDT (Current Medical Diagnosis & Treatment) 2007, Chapter 20, page 872
  15. ↑ Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN (2001). "Effect of infliximab on sight-threatening panuveitis in Behcet's disease". Lancet. 358 (9278): 295–6. doi:10.1016/S0140-6736(01)05497-6. PMID 11498218. 
  16. ↑ Sfikakis PP (2002). "Behcet's disease: a new target for anti-tumor necrosis factor treatment". Ann Rheum Dis. 61 Suppl 2: ii51–3. PMC 1766720 Freely accessible. PMID 12379622. 
  17. ↑ Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, Hamuryudan V, Yazici H (2005). "Short-term trial of etanercept in Behcet's disease: a double blind, placebo controlled study". J Rheumatol. 32 (1): 98–105. PMID 15630733. 
  18. ↑ Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, Basaran E (2002). "Interferon alfa-2a in the treatment of Behcet disease: a randomized placebo-controlled and double-blind study". Arch Dermatol. 138 (4): 467–71. doi:10.1001/archderm.138.4.467. PMID 11939808. 
  19. ↑ Kotter I, Zierhut M, Eckstein AK, Vonthein R, Ness T, Gunaydin I, Grimbacher B, Blaschke S, Meyer-Riemann W, Peter HH, Stubiger N (2003). "Human recombinant interferon alfa-2a for the treatment of Behcet's disease with sight threatening posterior or panuveitis". Br J Ophthalmol. 87 (4): 423–31. doi:10.1136/bjo.87.4.423. PMC 1771623 Freely accessible. PMID 12642304. 
  20. ↑ Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H (2002). "Interferon alfa combined with azathioprine for the uveitis of Behcet's disease: an open study". Isr Med Assoc J. 4 (11 Suppl): 928–30. PMID 12455182. 
  21. ↑ Yurdakul S, Mat C, Tuzun Y, Ozyazgan Y, Hamuryudan V, Uysal O, Senocak M, Yazici H (2001). "A double-blind trial of colchicine in Behcet's syndrome". Arthritis Rheum. 44 (11): 2686–92. doi:10.1002/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H. PMID 11710724. 
  22. ↑ Hamuryudan V, Mat C, Saip S, Ozyazgan Y, Siva A, Yurdakul S, Zwingenberger K, Yazici H (1998). "Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial". Ann Intern Med. 128 (6): 443–50. PMID 9499327. 
  23. ↑ Matsuda T, Ohno S, Hirohata S, Miyanaga Y, Ujihara H, Inaba G, Nakamura S, Tanaka S, Kogure M, Mizushima Y (2003). "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". Drugs R D. 4 (1): 19–28. doi:10.2165/00126839-200304010-00002. PMID 12568631. 
  24. ↑ Sharquie KE, Najim RA, Abu-Raghif AR (2002). "Dapsone in Behcet's disease: a double-blind, placebo-controlled, cross-over study". J Dermatol. 29 (5): 267–79. PMID 12081158. 
  25. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  26. ↑ Durrani K, Papaliodis GN (2008). "The genetics of Adamantiades-Behcet's disease". Semin Ophthalmol. 23 (1): 73–9. doi:10.1080/08820530701745264. PMID 18214795. 
  27. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  28. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  29. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
  30. ↑ Behcet's disease.
  31. ↑ Lua error in package.lua at line 80: module 'Module:Citation/CS1/Suggestions' not found.
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