What is Behçet's Disease?

It is a rheumatic disease that was first described by Turkish dermatology professor Hulusi Behçet and took its name from him. It is common in Turks. Although painful ulcerated sores in the mouth and genital area and inflammation of the eye, called uveitis, are the basic and diagnostic symptoms, Behçet's Disease is a vascular vasculitis that affects all organs and systems beyond these symptoms. The most important reason why the clinic is so colorful is that it covers all vessels, large, small, artery and vein. While it progresses with slightly milder clinical findings in women, the inflammation and associated clinical findings are severe in men. The severity of the clinic varies in each patient. In some, it progresses with wounds only in the mouth and genital area for years, while in others, it can be seen with lung, digestive system and brain involvement. It may always remain inflamed, or it may be seen as an attack and remission cycle in some patients.

Why?

It is more common in those with active HLA B5/B51 genetic locus. Although it is known that it is seen, pathogenesis studies continue today. It is known that inflammation in the vessels results from the deterioration of tolerance to the vessels in the immune system and the initiation of inflammation by the defense system. Environmental/epigenetic factors and hormones can pull the trigger. It is not a contagious disease and there is no absolute transmission between generations. It is common in countries on the historical Silk Road (Turkey, Iran, Asian countries and Japan). 20-30 years of age is the most common age range. Clinical findings and dermatological pathergy testing are important in diagnosis. However, rheumatology doctors who are experts on the subject are competent in diagnosing and treating Behcet's Disease.

Panuveitis in the eye (may lead to blindness), ulcerated sores in the mouth, rashes on the skin, painful ulcerated sores in the genital area, thrombosis and aneurysms in the veins, inflammation in the lung and Vascular occlusion, inflammation and vascular occlusion in the meninges, inflammation in the brain parenchymal tissue, ulcerated wounds along the intestines in the digestive system, involvement of the heart membrane and giant intracardiac clots are among the clinical findings that may be observed.

Treatment

Today, there is no treatment that will completely eliminate the disease. The goal of treatment is to eliminate inflammation and prevent any damage that may occur. It is intended to prevent. In this way, the deterioration of the person's quality of life is prevented and complications such as disability and blindness are tried to be stopped. Various medications are available. They are applied either topically on wounds, orally or intravenously, or into the skin, which we call subcutaneous. Steroids are also included in the treatment of this disease, and various disease-regulating antirheumatic agents and colchicine are also included in the treatment. Which medication to choose, how to apply it, and how long the treatment will last vary depending on the patient's condition. Since many organs and systems are involved, evaluation and treatment recommendations can be obtained from physicians of various branches with a multidisciplinary approach. However, the team leader, the doctors who will determine the follow-up scheme to diagnose the disease and arrange the treatment are internal medicine-rheumatology specialists. It should not be forgotten that even if the treatment is effective, exacerbations may occur.

 

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