Behcet Syndrome

Behcet Syndrome is a group of symptoms (that is, what you experience) and signs (that is, what the doctor finds by examining you). Doctors tend to talk of a syndrome rather than a disease when the cause linking the dif-ferent features is not known. This disorder of Behcet Syndrome is a protects the body against infections by producing controlled inflammation, becomes overactive and produces unpredictable out-breaks of unwanted and exaggerated inflammation. That is a chronic condition that happens because of disturbances in the body's immune system.

Behcet Syndrome is a chronic form of vasculitis (inflammation of the blood vessels) involving four primary. This is recurrent systemic disease characterized by uveitis with hypopyon, recurrent ulceration of the mucous membranes of the mouth and pharynx, and ulceration of the genitalia. That is central nervous system, heart, and intestinal tract may be involved. Onset is usually between 10 and 30 years of age, but may occur as late as age 45, and males are affected approximately 5 times more frequently than females. The effect of joint pain is a trait that usually comes later in the history of the disease than the ulcers of the mouth and genitalia. Recurrent every 2 to 3 months and lasting from one week to a month.

Behcet Syndrome protean manifestations often make early diagnosis difficult if classic features such as oral and genital ulcers are absent. The diagnosis is based on clinical features; there are no universally accepted diagnostic blood tests. The course is characterized by acute exacerbations (which generally abate in intensity over time) and periods of remission.

Causes of Behcet Syndrome

Some causes of Behcet Syndrome include :

• The cause of Behçet's syndrome is unknown, but viruses and autoimmune disorders may play a role.
• Cushings Syndrome is a condition caused by an excess of steroid hormones called cortisol. 
• Cortisol is sometimes called a stress hormone.
• Cortisol is vital for life as it helps to regulate blood pressure and the immune system .
• It helps the body respond to stress and balances the effect of insulin to keep blood sugar levels normal.

Symptoms of Behcet Syndrome

Common symptoms of Behcet Syndrome are:

• Almost everyone with this syndrome has recurring, painful mouth sores, similar to canker sores.
• A minor injury, even a puncture from a hypodermic needle, can cause the area to become swollen and inflamed.
• Patients who have a parent with Behçet syndrome have disease onset at a younger age (genetic anticipation).
• In addition, pediatric patients are more likely to have a family history of Behçet syndrome, compared to patients with disease onset as an adult.

Behcet Syndrome in Pregnancy

Several studies have examined the impact of pregnancy and the postpartum period on disease activity in Behcet Syndrome. A survey of women with Behcet Syndrome showed no significant difference in pregnancy outcomes as compared with healthy women and women with recurrent oral ulcerations. In a study of 44 pregnancies in 28 women with Behcet Syndrome, 52% went into remission during pregnancy; 20% had no change in their disease activity and 27% had an exacerbation. The most frequent flare-up manifested with an increase in intensity and severity of oral ulcerations. Flare-ups of genital ulcers, arthritis, and ocular inflammation were also seen. Overall it appears that pregnancy does not affect the natural course of the syndrome, and fetal outcomes are excellent.

Treatment of Behcet Syndrome

Despite few new agents being added to the therapeutic armamentarium, the overall prognosis for patients with Behcet Syndrome has improved over the past two decades. There are no controlled studies on the use of corticosteroids or anticoagulants in the treatment of Behcet Syndrome, but they are widely prescribed for various manifestations. Treatment options for ocular disease have been the most rigorously studied, and the incidence of significant loss of vision has decreased from 75% to 20% . Early identification and treatment is likely responsible for the decrease in morbidity. Because the pathogenesis (i.e., inflammatory versus thrombotic) of various manifestations of Behcet Syndrome varies, as does the extent of organ involvement (i.e., isolated manifestation versus systemic flare-up), the approach to treatment must be customized. The majority of clinical manifestations have been demonstrated to have vasculitis as an underlying etiology. Fevers are not characteristic of Behcet Syndrome and should be appropriately investigated for infections as their cause.

Some common methods for the treatment of Behcet Syndrome:

• The diagnosis is based on the physical examination because no laboratory tests can detect Behçet's syndrome.
• Although there is no cure, specific symptoms can usually be relieved by treatment. For example, a corticosteroid applied externally (rather than by mouth) can help heal inflamed eyes and skin sores.
• Cytotoxic medications are usually indicated in patients with ocular, CNS, and vascular disease. Biologic medications are also being used in patients with these complications. Decreasing morbidity and mortality is the goal of treatment for children with Behçet syndrome.
• Thalidomide Some Trade Names THALOMID
  is being investigated for use. Needle punctures should be avoided because the skin may become inflamed.
• Reiter's syndrome (reactive arthritis), Stevens-Johnson syndrome, systemic lupus erythematosus, Crohn's disease, herpes infection, and ulcerative colitis.

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