Introduction:

Behecet's is a chronic immune disease of unknown cause affecting multiple organs. The major signs of the disease are ocular inflammation (i.e. uveitis) and ,oral ulcers which can involve the lips, gums, mucous of the cheeks, pharynx , tongue and palate.  They are superficial and discrete and may persist for several days to several weeks. They have a gray-yellow surface and red rim. The lesions are painful and may interfere with eating and talking (Figure 1).Other sings can include genital ulcers, skin lesions and inflammation of the joints and blood vessels.

Accurate diagnosis typically based on the presence of these major signs at one time or another throughout the disease course. Ocular involvement occurs in 85-100% of patients and it is the second most common manifestation of disease after oral ulcers.

The highest incidence of Behcet disease is found in the Middle and Far East. It afflicts both sexes virtually all age groups and all races. The sings appear between 20-40 years old and are more common in males. There is a genetic predisposition to the diseases, and it is not contagious.

Major Signs:

Ocular signs can include severe inflammation in the front chamber of the eye( anterior chamber). This is called anterior uveitis. If the inflammation is severe enough, a layering of white colored cells can occur in the anterior chamber and disappears with treatment (Figure2). In the posterior part of the eye, the inflammation can involve the optic nerve and the retinal blood vessels. This inflammation can cause atrophy of the optic nerve and the affected area Figure (3, 4).Other ocular manifestations can include "complicated cataract", secondary glaucoma, and eventual phthisis (shrinking of the eye).

Genital lesions occur more frequently in women than in men. They typically involve the vagina or scrotum and are similar in appearance and description to the oral lesions. These ulcers normally disappear leaving no scars. The testes can become painful and swollen. Epididymitis is a major sing of the disease.

Skin lesions often resemble acne. They can also appear as a larger, tender elevated nodule. They can ulcerate or spontaneously disappear.

An inflammation of the veins in the skin may also occur. This sometimes can occur spontaneously at the site of a needle injection or skin injury.

Approximately 50% of Behcet patients experience a non-erosive arthritis, with the big joints, especially the knee, being the most common joint affected. Other affected areas include the ankles, elbows and wrists. Arthritis is often linked to an active stage of the disease elsewhere in the body.

Involvement of the gastro-intestinal tract is common and can cause ulcerative colitis. This is characterized by abdominal pain, flatulence, diarrhea or constipation, and bleeding from the rectum which can be painful.

Mild renal impairment occurs in about 20% of patients.

Inflammation of the blood vessel walls can weaken the wall and cause an aneurysm or thrombosis which can lead to infarction or gangrene of the bowel and an aneurysm. Although this complication is infrequent, it is serious.

Central nervous system involvement may occur in advanced Behcet which may lead to brain thrombosis.

Disease Course:

Behcet disease is characterized by attacks (exacerbation) and remissions. These attacks may come on slowly or very quickly and cannot be predicted. Signs in any part of the body can appear at different times, chronically and/or intermittently. Thus long-term follow-up is required for the patient even after the diagnosis is made and the disease appears inactive. The best visual results will be obtained if medical treatment is started at the first signs of an attack. For this reason, it is important to seek immediate medical care.

Generally, there is little or no warning that an attack is about to occur. There are no clearly recognized agents that will cause an exacerbation; however, emotional or physical stress have been reported to proceed an attack. Patients may have 4-5 attacks per year but with time, the attacks may become less frequent and may occur only once or twice a year. Any sudden deterioration or decrease in the visual acuity requires immediate visit to the emergency room, even if the patient has a coming appointment with the physician.      

Treatment:

Thus far, as the major cause of Behcet disease is still unknown the disease cannot be completely cured. Medications are aimed at suppressing inflammation and preventing serious complications such as blindness and disability.  Corticosteroidsare used in the acute phase but for long-term are not required or desired. Patients with severe ocular involvement require treatment with immunosuppressives such as cyclosporine, azathiprine, methotrexate, mycophenolate mofetil and infleximab. These agents may be used for one year or more. In some cases, colchicines may be used to prevent re-occurrence.  Treatment is aimed at the acute sings and at preventing recurrence. While ocular and skin symptoms tend to respond well to medications, joint pains may respond to non-steroidal anti-inflammatory drugs such as ibuprofen.

Side Effects of Behcet Medications:

Long-term use of cortisone medications may cause diabetes, hypertension, osteoporosis, cataract or glaucoma. In addition, long-term use of  immunosuppressives weaken the immune system and can cause renal or hepatic dysfunction, poor production of marrow white blood cells and potential hypertension.

Don’t use any medication without consulting your doctor.

Discontinuing medication without your doctor's instruction may lead to an exacerbating  and difficult-to-manage disease. It is important to strictly follow the instruction of the doctor and the pharmacist.

It is important to do lab examinations and to regularly check the blood pressure. It is also recommended to take calcium supplements if cortisone medications are to be used for long term. 

Revised Feb 2012