Behçet's disease
From Wikipedia, the free encyclopedia
| 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) is a chronic condition due to disturbances 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.
Contents |
[edit] 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.[1][2] 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.[3] Its first modern formal description was published in 1922.[1]
Some sources use the term "Adamantiades’ syndrome" or "Adamandiades-Behçet syndrome", for the work done by Benediktos Adamantiades.[4] However, the current World Health Organization/ICD-10 standard is "Behçet's disease".
In 1991, Saudi Arabian medical researchers described "neuro-Behcet's disease",[5] a neurological involvement in Behcet's disease, considered one of the most devastating manifestations of the disease.[6]
[edit] 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)
[edit] 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).
[edit] 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 personality, psychoses,
- any other members of the family with a diagnosis of Behçet disease.
[edit] '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.
[edit] 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 definitively hereditary but 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. ( http://www.nlm.nih.gov/medlineplus/behcetssyndrome.html )
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, study has revealed a connection to food allergy, particularly to dairy products.[7] Dramatically successful treatment has been reported in one case of Behçet disease in which the patient had many food allergies detected through ELISA testing for IgE and IgG antibodies.[8]
[edit] 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.[9][10] Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms.[11]
Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers[12] as well as ocular lesions.[13] Azathioprine, when used in combination with interferon alfa-2b also shows promise,[14] and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis in women, and arthritis in men.[15]
Thalidomide has also been used due to its immune-modifying effect.[16] Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions.[17][18]
A different orientation could be explored in Behçet Disease, especially with genetic linkage to HLA-B51 antigenOhno, S.; M. Ohguchi, S. Hirose, H. Matsuda, A. Wakisaka, M. Aizawa (1982). "Close association of HLA-Bw51 with Behcet's disease". Archives of Ophthalmology 100 (9): 1455–1458., 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.[citation needed]
[edit] 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.
[edit] Pronunciation note
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".
[edit] References
- ^ a b synd/1863 at Who Named It
- ^ 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.
- ^ Johns Hopkins Vasculitis Center (2004). Johns Hopkins Vasculitis Center Discusses Behcets Disease. Retrieved September 9, 2005.
- ^ B. Adamandiades. Sur un cas d'iritis à hypopyon récidivant. Annales d'oculistique, Paris, 1931, 168: 271-278.
- ^ Ravi Malhotra (2004), "Saudi Arabia", Practical Neurology 4: 184-185.
- ^ S. Saleem (2005), Neuro-Behcet's Disease: NBD, Neurographics, Vol. 4, Issue 2, Article 1.
- ^ Triolo et. al. (2002). Humoral and cell mediated immune response to cow's milk proteins in Behçet's disease. Ann Rheum Dis. 2002 May;61(5):459-62.
- ^ Center For Food Allergies, http://www.centerforfoodallergies.com/Behcet.htm.
- ^ 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:. PMID 11498218.
- ^ 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.
- ^ 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.
- ^ 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:. PMID 11939808.
- ^ 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:. PMID 12642304.
- ^ 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.
- ^ 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:. PMID 11710724.
- ^ 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.
- ^ 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:. PMID 12568631.
- ^ 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.
[edit] External links
- Behçet's Disease Resource Guide from the National Eye Institute (NEI).
- Behcet disease - MedLink Neurology Clinical Summary
- Signs & Symptoms of Behçet's disease (with pictures)
- Behçet’s disease: from Hippocrates to the third millennium PDF British Journal of Ophthalmology (2003)
- American Behçet's Disease Association
- Behçet's Syndrome Society (UK)
- International Society for Behçet's Disease
- DermNet systemic/behcet
