Behcet's Syndrome (cont.)

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How is Behçet's syndrome diagnosed?

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Behçet'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 Behçet's syndrome. (The other criteria above are still required for ultimate diagnosis.) This test consists of pricking the skin of the forearm with a sterile needle. The test is called positive and suggests Behçet'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 after the test.

Tests such as skin biopsy, lumbar puncture, MRI scan of the brain, and bowel tests are considered for individual patients based upon symptoms that are present.

What is the treatment of Behçet's syndrome?

The treatment of Behçet's syndrome depends on the severity and the location of its manifestations in an individual patient.

Steroid (cortisone) gels, pastes (such as Kenolog in Orabase) and creams can be helpful for the mouth and genital ulcers. Colchicine (Colcrys) can also minimize recurrent ulcerations. Trental (pentoxifylline) has also been used in the treatment of oral and genital ulcers.

Joint inflammation can require nonsteroidal 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 (Azulfidine) has been effective in some patients for arthritis. Bowel disease is treated with oral steroids and sulfasalazine.

Diligent treatment of eye inflammation is essential. Patients with eye symptoms or a history of eye inflammation should be monitored by an ophthalmologist. Resistant eye inflammation can often respond to new biologic medications that block a protein that plays a major role in initiating inflammation, called TNF. These TNF-blocking medications, including infliximab (Remicade) and adalimumab (Humira), can also be helpful for severe mouth ulcerations.

Severe disease of the arteries, eyes, and brain can be difficult to treat and require powerful medications that suppress the immune system called immunosuppressive agents. Immunosuppressive agents used for severe Behçet's syndrome include chlorambucil (Leukeran), azathioprine (Imuran), and cyclophosphamide (Cytoxan). Cyclosporine has been used for resistant disease.

Studies suggest that thalidomide (Thalomid) may be of benefit for certain patients with Behçet's syndrome in treating and preventing ulcerations of the mouth and genitals. Side effects of thalidomide include promoting abnormal development of fetal growth, nerve injury (neuropathy), and hypersedation. Trials are currently underway evaluating interferon alpha for the treatment of eye disease in patients with Behçet's syndrome.

Medically reviewed by Kirkwood Johnston, MD; American Board of Internal Medicine with subspecialty in Rheumatology
REFERENCES:

American College of Rheumatology, Annual Scientific Meeting; November, 2006, 2007.

Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.

Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.


Medically Reviewed by a Doctor on 12/11/2013

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