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Sep 05, 2006
Behcet's Syndrome
by SirGan
Not too many people have heard about the Behcet's disease or Behcet’s syndrome because it is one specific and extremely rare, chronic inflammatory disorder. It is important to point out that the cause of Behcet's disease is unknown, although there have been reports of a virus found in some individuals with the disease. Researches done in the past have shown that Behcet's disease generally begins when individuals are in their 20s or 30s, although it can happen at any age. It tends to occur more often in men than in women. Most common symptoms of this condition are these recurrent ulcers in the mouth and on the genitals, and eye inflammation. The most important thing there is to know about this condition is that it represents a multi-system disease because it may involve all organs and affect the central nervous system, causing memory loss and impaired speech, balance, and movement. That’s why- it is considered to be a very serious condition because -the effects of the disease may include blindness, stroke, swelling of the spinal cord, and intestinal complications. Although there is no cure for this disease- incurable does not mean untreatable. There are several ways in which the immune system can be suppressed to reduce the extra inflammation, and this relieve the symptoms.
Who’s affected with this condition?Although Behcet's can affect someone of any age and has been diagnosed in young infants and people over the age of 70, it most commonly affects people between the ages of 20 and 40.
In Western Europe and North America, more women are affected than men. No-one knows for sure how many sufferers there are in the UK but it is estimated that there are about 2 in 100,000, that is, about 2000 people. The disease is common in Japan, Turkey and Israel, and less common in the United States.
Historical factsResearches done in the past have confirmed that, in 1937, Hulusi Behcet, a Turkish dermatologist, described a disease characterized by inflammation of the uvea, the middle coat of the eye comprising the choroids, ciliary body, and the iris with genital and oral ulcers.
Possible causes of the conditionUnfortunately- life for Behcet's sufferers is made more difficult because of misunderstandings about the illness. The most common misconception is that the condition is infectious and sexually transmitted because of ulcers on the mouth and genitals- but the fact is that it is not! The cause of this uncommon condition remains unknown, but experts in the field of Behcet's research agree that it may be genetic.
The cause of Behcet's disease is unknown. Most experts are contemplating about possible genetic predisposition, autoimmune mechanisms, and viral infection as a possible cause of the disease! These possible causes may trigger the autoimmune process, causing the body to attack its own blood vessels, making them inflamed.
Symptoms of Behcet’s diseaseEvery patient should know that the symptoms of Behcet's syndrome depend on the area of the body affected because it may involve inflammation of many areas of the body. When we talk about inflammation target tissue then we should know that these areas include the arteries that supply blood to the body's tissues and veins that take the blood back to the lungs. The most common sights of inflammation include:
Patients should know that the mouth and genital ulcers are generally painful and can range in size from a few millimeters to 20 millimeters in diameter. The mouth ulcers occur on the gums, tongue, and inner lining of the mouth. The genital ulcers occur on the scrotum and penis of males and vulva of women and can leave scars.
EyesThis is very serious symptom of this disease because an inflammation of the eye can lead to blindness. Symptoms of eye inflammation include:
ArteriesIt is extremely important to know more about the inflammation of arteries as a possible complication because if the arteries become inflamed in patients, it can lead to death of the tissues whose oxygen supply depends on these vessels.
BrainSymptoms of inflammation of the brain or meninges include:
This could be one very serious complication because inflammation of the brain and the meninges can cause very serious damage to nervous tissue and lead to weakness or impaired function some parts of the body. The worst possible scenarios is developing of confusion and coma.
JointsIf we talk about the joints affection then we should know that knees, wrists, ankles, and elbows are the most common joints affected. Several symptoms occur as a complication of joint inflammation and the most commons are:
SkinIt is important to point out that sometimes the symptoms are also present on the skin of the patients! The skin can develop areas of inflammation which appear as raised, tender, reddish, typically on the front of the legs. The worst scenario is appearing of ulcerations which can occur at any location in the stomach, large or small bowel.
Diagnosis of Behcet’s disease
Behcet's syndrome is diagnosed based on the finding of recurrent mouth ulcerations combined with any two of the following: eye inflammation, genital ulcerations, or skin abnormalities mentioned above.
A special skin test called a pathergy test can also suggest Behcet's syndrome. This test consists of pricking the skin of the forearm with a sterile needle. The test is called positive and suggests Behcet's syndrome when the puncture causes a sterile red nodule or pustule that is greater than two millimeters in diameter at 24 to 48 hours.
Other diagnostic tools which can help in setting the right diagnosis:
Treatment of Behcet’s diseaseEvery patient should know that there is no definitive cure for the Behcet syndrome- there is only symptomatic treatment! The treatment of Behcet's syndrome depends on the severity and the location of its manifestations. Good thing is that several classes of medications have showed the potential to relieve some of the symptoms! Some of the most commonly used are:
Steroid gels, pastes and creams (in most cases we are talking about cortisone) can be helpful for the mouth and genital ulcers because these steroids are suppressing the normal or increased inflammatory response of our body! Colchicine can also minimize recurrent ulcerations and Trental seemed to maintain the healed ulcers for up to the 29 months.
Joint inflammation can require non-steroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine has been effective in some patients for arthritis.
It is extremely important that the treatment of eye inflammation is essential and that’s why- patients with eye symptoms should be monitored by an ophthalmologist all the time.
There is no doubt- severe disease of the arteries, eyes, and brain can be extremely difficult to treat. That’s why, in order to do this- treatment requires powerful medications that suppress the immune system. These medications are called immunosuppressive agents. Most commonly used immunosuppressive agents include chlorambucil (Leuderan), azathioprine (Imuran), and cyclophosphamide (Cytoxan).
Almost everyone has heard about the famous medication called Thalidomide! Recent studies suggest that thalidomide may be of benefit for certain patients with Behcet's syndrome in treating and preventing ulcerations of the mouth and genitals. It is crucial to point out that this medication is famous by its side effects and the most commons are: promoting abnormal development of fetal growth, nerve injury (neuropathy), and hypersedation.
Prognosis of Behcet’s syndromeUnfortunately- the prognosis for Behcet's syndrome is generally poor. There has been a documented case of Behcet's lasting for 17 years. Every patient should know that, although the disease is considered painful but not fatal, the fact is when the central nervous system is involved there is usually severe disability and death often occurs. The condition is usually chronic, although there can be remissions during the course of the disease.
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