Take-Home Messages from ACR 2020: Behcet’s Disease



Alvin Wells, MD, PhD: One of the nice things that was presented at the ACR [American College of Rheumatology Convergence] meeting were data on an oral agent for patients with psoriatic arthritis and Behcet disease. Behcet disease is a chronic systemic disorder that can affect a number of areas of the body. The classic symptoms are oral and genital ulcers. In addition, we actually see the disease can have an impact on the eyes. It can actually cause some arthritis. Indeed, in patients with severe disease, it can actually involve the central nervous system [CNS] and the peritoneal system.

Before we were looking at drugs such as apremilast, used to treat the oral ulcers in patients with Behcet disease, the only thing we had to offer our patients was high doses of prednisone. Despite that, some people still had significant impact of disease, oral ulcers that would respond to only 30 or 40 mg of prednisone or more.

Data revealed that patients with active oral ulcers treated with apremilast saw not only significant improvement and healing of the ulcers but also pain improved. That really struck me at last year’s meeting. And that pain improvement happened very rapidly—within a week for many patients.

The take-home message here: For a chronic systemic disease like Behcet disease that causes significant oral ulcers and that’s recalcitrant to prednisone, agents such as apremilast can have a dramatic impact. They are going to be a tool that I’ll use in my clinic to treat patients who have Behcet disease.

There are some data coming out of a French group. They’re looking at patients who have recalcitrant Behcet disease. Remember, this is a rare autoimmune disease that mostly causes oral and genital ulcers. But some people have uveitis or CNS disease. And in many of these patients, corticosteroids and other drugs are inadequate.

They did a study with a small number of patients—there were only 10 patients on the trial. They said, “Let’s look at autologous hematopoietic stem cells to see if they can provide some improvement.” These patients were on their background drugs, including corticosteroids and methotrexate. In a significant number of these patients—6 of 10 patients—there’s significant improvement, almost remission of disease.

At the end of the day, they looked at the safety profile too. Over a period of time, investigators did not see any new safety signals that we did not expect in patients having stem cell transplants. You still want to look for abnormal infections and viral disease. However, nothing was reported from this short trial.

The nice thing about this is that for a group of patients whose disease is not controlled by a certain medication that we use on a daily basis, including methotrexate and high doses of corticosteroids, autologous hematopoietic stem cell transplant might be an option. However, this is something that needs to be studied in more phase 2 and phase 3 clinical trials.

When I go to the ACR meeting, I’m always looking for things that are new, that are on the horizon. For Behcet disease, a couple of things were highlighted at last year’s meeting. We already chatted about the potential use of autologous hematopoietic stem cells. That’s in early stages, but it is something out there as well.

Can we use other agents? We talked about apremilast. Are there other PDE4 inhibitors and maybe some other new mechanisms of action? We know that some of the other cytokines—IL-23 and even IL-17—may play a role. You’re beginning to see a snapshot here. In some small centers, some of these agents are being tested in early phase 1 and phase 2 clinical trials.

In this group of patients who don’t always respond to methotrexate, who don’t respond to corticosteroids, having another treatment option to help get the disease under control and maybe prevent progressing would be exciting. We look forward to seeing more of this information emerge at our future ACR meetings.

Transcript Edited for Clarity

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