Other Nemes: Behçet’s syndrome, Morbus Behçet, Behçet-Adamantiades syndrome, or Silk Road disease.
Definition:
Behcet’s disease (BD), is a chronic form of vasculitis (inflammation of the blood vessels) involving four primary symptoms: oral and genital ulcers, ocular inflammation, and arthritis.
The inflammation of Behcet’s disease leads to numerous symptoms that may initially seem unrelated. The signs and symptoms of Behcet’s disease — which may include mouth sores, eye inflammation, skin rashes and lesions, and genital sores — vary from person to person and may come and go on their own.
The exact cause of Behcet’s is unknown, but it may be an autoimmune disorder, which means the body’s immune system mistakenly attacks some of its own healthy cells. Both genetic and environmental factors may be responsible for Behcet’s disease.
Description:
Behçet’s disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis. As a systemic disease, it can also involve visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, cardiovascular and neurological systems. This syndrome can be fatal due to ruptured vascular aneurysms or severe neurological complications.
In the 1930s Hulusi Behçet observed the three classic symptoms (oral and genital ulcers and eye inflammation) now define this complex condition. BD also has a unique ability to affect all sizes of blood vessels, including arteries and veins. Symptoms related to vasculitis, such as inflammation of joints, gastrointestinal areas, or the central nervous system, are also common.
Symptoms of this disease may have been described by Hippocrates in the 5th century BC, in his 3rd Epidemion-book. Its first modern formal description was published in 1922.
Some sources use the term “Adamandiades syndrome” or “Adamandiades-Behçet syndrome”, for the work done by Benediktos Adamantiades. However, the current World Health Organization/ICD-10 standard is “Behçet disease”.
In 1991, Saudi Arabian medical researchers discovered “neuro-Behcet’s disease”, a neurological involvement in Behcet’s disease, considered one of the most devastating manifestations of the disease
Demographics:
Incidence of BD is very rare in the United States with approximately five in 100,000 people developing the syndrome. In Middle Eastern and Asian countries between Iran and Japan (known as the “Old Silk Route”), BD is quite prevalent. Incidence in these countries is double that of the United States.
More than twice as many females are diagnosed with BD than males in the United States. However, in Middle Eastern and Asian areas, significantly more men are affected than females.
Causes:
No one knows why the immune system starts to behave this way in Behçet ‘s 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 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.
Behçet disease is normally caused by an autoimmune response that triggers inflammation of the blood vessels. Researchers have discovered a gene, HLA-B51, which predisposes an individual to BD. However, not all individuals with this gene develop the disease. The specific event leading to onset of BD is not known, but there are speculations that it may be related to the following:
*herpes simplex virus infections
*frequent infections of Streptococcus bacteria
*environmental factors
Diagnosis & Symptoms:
Behçet disease is diagnosed based on a set of guidelines established by an international group of physicians. A physician observes clinical signs and symptoms during patient examination. The most recent and accepted guidelines for a positive diagnosis include the presence of recurring oral ulcers (three or more times in one year) and at least two of four secondary symptoms, including recurring genital ulcers, uveitis, skin lesions, a positive pathergy test.
A pathergy test is a skin-prick test to see if a red bump will form at the injection site. If there is a reaction, the test is positive. This test may be given to patients suspected of BD, but it is not an indicator for the disease. Only a small percentage of patients diagnosed with BD actually test positive.
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 (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
*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 Behcet disease
Mouth ulcer with Behcet’s Disease.
Aphthous ulcer in a patient with Behcet Disease.
…. .
Aphthous ulcer in a patient with 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.
CLICK TO SEE THE TESTS FOR DIAGNOSIS
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. Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms.
Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers as well as ocular lesions. Azathioprine, when used in combination with interferon alfa-2b also shows promise, and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis in women, and arthritis in men.
Thalidomide has also been used due to its immune-modifying effect. Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions.
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.
Prognosis:
For most patients, the prognosis of Behçet disease is good. Individuals typically experience periods of active symptoms followed by periods of remission in which there are no symptoms. The length of these intervals varies, with ulcerous outbreaks lasting a few weeks and other symptoms occurring for longer durations. With proper treatments and medication, patients can continue to lead active lifestyles in most cases.
Development of vascular or neurological complications often indicates a poorer prognosis. Blindness due to ocular inflammation is also prevalent in patients with BD.
Recovery and rehabilitation:
Unlike most diseases, BD has symptoms that periodically flare up and then disappear for a period of time. As a result, patients may have long intervals with no complications. After treatment for active symptoms, patients usually require rest due to fatigue. Moderate exercise is also recommended to improve circulation and muscle strength.
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Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose
Resources:
http://en.wikipedia.org/wiki/Behcet%27s_disease
http://www.healthline.com/galecontent/behet-disease
http://www.visualsunlimited.com/browse/vu425/vu425957.html
http://vasculitis.med.jhu.edu/typesof/behcets.html
http://www.visualsunlimited.com/browse/vu425/vu425958.html
