Currently recommended treatment of Behçet disease

What is the currently recommended treatment of Behçet disease?

Gülen Hatemi, MD: The treatment of Behçet syndrome should be individualized according to the sex and age of the patient and types of organ involvement.

For patients who have only mucocutaneous and joint involvement, usually we start with colchicine, and many patients benefit from this treatment. Topical corticosteroids and topical antibiotics for genital ulcers can also be used. In patients who are refractory to colchicine, azathioprine, interferon alpha, and tumor necrosis factor (TNF)-alpha blockers can be tried. Apremilast is a more recently used agent for Behçet syndrome that also works quite well for oral ulcers.

For the management of major organ involvement—meaning uveitis, vascular involvement, gastrointestinal and nervous system involvements—it is necessary to use immunosuppressives right from the start. It is very important to suppress the attacks quickly, so that they do not leave damage. Corticosteroids may be used for a short period during the attack. In order to prevent the next attack, we need to use immunosuppressives. We usually start with azathioprine. In uveitis we may add cyclosporine to these and use interferon alpha or TNF-alpha blockers in resistant cases.

In pulmonary artery aneurysms, cyclophosphamide is usually the first-line agent, since artery aneurysms can be lethal in these patients. We usually give 6 pulses of IV cyclophosphamide together with high-dose corticosteroids and afterwards continue with azathioprine maintenance. TNF-alpha blockers can also be used in resistant cases.

For deep vein thrombosis, we usually start with azathioprine. In resistant cases, interferon alpha or anti-TNFs can be used. There is some controversy regarding the use of anticoagulants in Behçet syndrome. Some centers do prefer to use them, and some centers do not. If you decide to use anticoagulation in Behçet syndrome patients, you have to make sure that they do not have any aneurysms, because anticoagulation can cause lethal bleeding in such patients.

The management of nervous system involvement is again with azathioprine as first-line therapy, and interferon alpha, TNF-alpha blockers, and interleukin (IL)-6 blockers in refractory cases.

For gastrointestinal involvement, the management is very similar to Crohn disease. In mild cases we start with 5-aminosalicylic acid (ASA) derivatives, and in moderate to severe cases, with azathioprine. In patients who are refractory to azathioprine, thalidomide or TNF-alpha blockers may be used.