Behçet's disease

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Behçet disease
ICD-10 M35.2
ICD-9 279.4
OMIM 109650
DiseasesDB 1285
eMedicine med/218  ped/219 derm/49 oph/425
MeSH D001528

Template:Behcet's disease For patient information click here Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Cafer Zorkun, M.D., Ph.D. [2] Synonyms and keywords: Behçet's syndrome; Morbus Behçet; silk road disease

For the heart in Behçet's disease click here

Overview

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 infections 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 vessels, 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.

History

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.[1] The name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.

A group of similar symptoms were first described by Hippocrates in 5th century BC as part of his 3rd Epidemion book.[2]

Pathology

The symptoms of Behçet's 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,genital ulcers, 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, intestinal complications, stroke, and even meningitis, which can be fatal.

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 remissions and exacerbations which can last from days to months. Complete remission is rare.

Diagnosis

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.

There are three levels of certainty for diagnosis:

  1. International Study Group diagnostic guidelines (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

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

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

'Suspected' or 'Possible' 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.

Causes

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 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.

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 patients with genital and oral ulcers[6] as well as with ocular lesions.[7] Azathioprine, when used in combination with interferon alfa-2b also shows promise,[8] and Colchicine may also be useful for treating some genital ulcers, erythema nodosum, and arthritis.[9]

Thalidomide has 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 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.

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. ↑ Template:WhoNamedIt
  2. ↑ Johns Hopkins Vasculitis Center (2004). Johns Hopkins Vasculitis Center Discusses Behçets Disease. Retrieved September 9, 2005.
  3. ↑ 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. PMID 11498218.
  4. ↑ Sfikakis PP (2002). "Behcet's disease: a new target for anti-tumor necrosis factor treatment". Ann Rheum Dis. 61 Suppl 2: ii51–3. PMID 12379622.
  5. ↑ 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.
  6. ↑ 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. PMID 11939808.
  7. ↑ 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. PMID 12642304.
  8. ↑ 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.
  9. ↑ 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. PMID 11710724.
  10. ↑ 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.
  11. ↑ 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. PMID 12568631.
  12. ↑ 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.

External links

Template:Diseases of the musculoskeletal system and connective tissue

de:Morbus Behçet he:תסמונת בכצ'ט nl:Ziekte van Behçet


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