Behcet's Syndrome (cont.)
How is Behcet's syndrome diagnosed?
Behcet'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 Behcet'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
Behcet'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.
Tests such as skin biopsy, lumbar puncture, MRI scan of the
brain, and bowel tests are considered based upon symptoms that are
present.
What is the treatment of Behcet's syndrome?
The treatment of Behcet'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 can also
minimize recurrent ulcerations. Mouth and genital ulcers healed and were reported at a national meeting of the American College of Rheumatology as less frequent in
nine or 12 patients who were treated with Trental (pentoxifylline). Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients.
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. Recent research has reported successful management of resistant eye inflammation with 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 etanercept (Enbrel), 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
Behcet's syndrome include chlorambucil (Leukeran), azathioprine (Imuran),
and cyclophosphamide (Cytoxan). Cyclosporine has been used for
resistant disease.
Recent studies suggest that thalidomide may be of benefit for
certain patients with Behcet'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.
Recent research further suggests that patients who have had
heart-valve replacement because of severe damage as a result of
inflammation can benefit by immune suppression with azathioprine and
prednisone after operation.
Trials are currently underway evaluating interferon alpha for the
treatment of eye disease in patients with Behcet's syndrome.
- Behcet's syndrome is associated with inflammation of
various areas of
the body.
- Symptoms of Behcet's syndrome depend on the body areas
affected.
- Recurrent mouth ulcers are characteristic of Behcet's
syndrome.
- Treatment of Behcet's syndrome depends on the severity and
the
location of its manifestations.
References:
Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.
Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.
American College of Rheumatology, Annual Scientific Meeting; November, 2006, 2007.
Last Editorial Review: 2/25/2008
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