Clinical Associate Professor of Medicine
Department of Rheumatology
New York University School of Medicine
New York, New York
Yusuf Yazici, MD, has disclosed that he has received funds for research support from Bristol-Myers Squibb, Celgene, and Genentech and consulting fees from Celgene and Sanofi.
When I plan treatment for my patients with Behçet’s disease, my immediate concern is to determine the extent and severity of disease, as treatment options differ depending on the systems involved.
Mucocutaneous involvement, including oral and genital ulcers and skin lesions such as acne and nodular lesions, can generally be treated with one group of medications, whereas more severe manifestations, such as those seen in the eye, vascular system, or central nervous system, are treated with another set of medications.
The goal of treatment is to control all symptoms early to prevent lasting damage. A multidisciplinary approach may be appropriate for some patients who have multiple organ system involvement. Later in the disease course, because the disease tends to get milder as time passes, there is a real chance of tapering medications and keeping patients in remission with little need for drug therapy. For these patients, we are able to taper or stop medications after 2-5 years of treatment.
Controlling Oral Ulcers and Other Mucocutaneous Lesions
Most patients with Behçet’s disease present with mucocutaneous manifestations, including oral ulcers. These oral ulcers can become very difficult to control and have been shown to have a major impact on quality of life.
Until recently, there were no approved medications specifically for Behçet’s disease. Most patients were treated with colchicine as a first-line drug; response time to this drug is typically short and it is effective for oral ulcers and skin lesions in some patients only.
Other treatments that have been tried include dapsone and pentoxifylline, both with some success resolving oral ulcers in patients with Behçet’s disease, and thalidomide, which has been used for oral and genital ulcers and follicular lesions.
For more advanced disease, other more immunosuppressive options have been tried, such as azathioprine, interferon-2α, and TNF-α inhibitors.
Apremilast, an oral phosphodiesterase‑4 inhibitor, was approved earlier in 2019 for the treatment of oral ulcers in patients with Behçet’s disease and is now part of our treatment algorithm based on its very good efficacy and safety data.
For patients with predominant mucocutaneous involvement with oral ulcers, apremilast should be considered either after colchicine has been tried or as first-line treatment.
In patients who do not respond to apremilast (before or after colchicine), the recommendation is to move to additional options, such as azathioprine, to control the active mucocutaneous disease.
Other treatment options are on the horizon. Biologic agents currently under investigation for the treatment of Behçet’s disease include secukinumab and ustekinumab; these are monoclonal antibodies targeting the IL-17 and the IL‑12/23 pathways, respectively. Both are in early phases of investigation but there are data that seem promising.
I’d like to hear from you. How are you currently treating patients with Behçet’s disease? Do you have experience with apremilast? I encourage readers to post their thoughts and questions in the comments section below.