SYNDROME OR BEHCET´S DISEASE

This topic was talk of a rare disease called BEHCET.

 In 1937, Hulusi Behçet Turkish doctor described the syndrome is characterized by three symptoms: aphthous stomatitis, genital ulcers and uveitis,inflammation of the capillaries that supply the eye located in the uvea.

Dr.Hulusi Behçet

Behcet's syndrome (also Behçet) is a chronic rheumatic disease that causes inflammation of blood vessels (vasculitis) of unknown cause that can affect almost any part of the body (widespread or systemic distribution) and is listed as a rare disease .

It is an autoimmune disease where the immune system attacks the hair causing inflammation. The origin is unknown but is believed to appear in genetically predisposed individuals who are exposed to some external agent, probably an infection (a bacterium), but usually begins between twenty and forty years, but may occur at any age.

The disease is most prevalent in Mediterranean countries and Asia, but its distribution is universal. Patients with the disease tend to have defects in the immune system, whose function is to defend the body against infectious agents such as viruses and bacteria.

Behçet's disease has a very low incidence and a chronic course.

Its main features are:  

• ulcers in the mouth in the form of painful sores.

• Genital ulcers and ocular inflammation.

• It can also cause various types of skin lesions.
• Inflammation of the joints (arthritis)

• intestinal inflammation with diarrhea

• inflammation of the nervous system, both central (brain, cerebellum, brainstem, spinal cord, meninges) and peripheral nerves (arms and legs).

These injuries are the result of inflammation of arteries and veins.The development of Behçet's disease is often intermittent, with periods of remission (no disease activity) and exacerbation (period’s activity) over the years, with a progressive tendency toward remission. Symptoms can last from days to weeks or may persist for
months or years. Often lead to disability that diminishes the quality of life.

In 1990 , the International Study Group for Behçet's Disease published a classification system to diagnose a patient and requires the presence

From:

• Repeat oral ulcers plus two or more of the following.

 • Genital ulcer recurrence.

 • Inflammatory lesions of the eye.

 • Specific skin lesions.

• Positivity of a test that measures the reactivity of the skin (pathergy).

Other manifestations of the disease which may be useful for diagnosis in individual cases are:

• subcutaneous thrombophlebitis.

• Deep vein thrombosis.

• Arterial obstruction, arterial embolism or aneurysm (ballooning of the artery in a sac).

• Neurological symptoms.

• Arthritis.

• Gastrointestinal symptoms.

• History of family history.

The disease can affect different organs or body systems. Many of the symptoms are similar to other symptoms of other diseases, which may mislead the physician. Since there is no fully tested feature of the disease, its diagnosis is clinical, based in symptoms and signs presented by the patient. It is necessary to rule out other possible causes before confirming the final diagnosis. It may sometimes be overdiagnosed, if there is excess or suspected misuse of the criteria. The more trained doctor for initial diagnosis of this disease is rheumatologist, and the U.S. American rheumatologist is a physician "header" of these patients.

Specialists are often manifestations of this disease:

• Rheumatologist, for diseases of the joints, or systemic manifestations triggered by vasculitis.

• Ophthalmologist for eye injuries.

• Dermatologist for skin lesions.

• Gynecologist, for genital ulcers and cervix in women.

• Gastroenterologist, related to the gastrointestinal tract.

• Neurologist, for matters related to the central nervous system.

• Psychologist to treat adjustment disorders caused by the disease.

If you have trouble finding doctors with knowledge of Behçet's disease contact the League of rheumatic patients, with the Spanish Society of Rheumatology or your local rheumatologist.

You can also request information from the American Association for the disease:

American Association for Behçet's Disease

1671 Summit Avenue

St. Paul, MN 55105-1815, U.S.A.

Tel: 1 - (800) 7BEHCETS or 1 (800) 723-4238

http://www.behcets.com

http://pw2.netcom.com/ ~ mharting / behcet.html

As the cause of the disease is unknown, treatment is done according to individual symptoms and the time of onset. Medications aim to reduce inflammation or to attempt to regulate the immune system. Some of the medicines used are:

• Local corticosteroids, which can be applied directly to skin lesions, mouth or eyes to reduce swelling and pain.

• Nonsteroidal anti-inflammatory and pain medications such as aspirin, ibuprofen and acetaminophen to relieve joint inflammation and pain.

 • Colchicine, used in the treatment of thrush.

• Oral corticosteroids such as prednisone, to reduce inflammation.

 • Other drugs used in some cases are pentoxifylline and dapsone.

• Immunosuppressive drugs, which are treatments that attempt to curb the immune activity. Are reserved for severe disease manifestations and require strict monitoring of treatment. We used azathioprine, cyclosporine, tacrolimus, cyclophosphamide, methotrexate and interferon alfa.

Now receive thalidomide or Talizer.

              This disease is not contagious.