Apremilast in Refractory Behçet's Syndrome: A Multicenter Observational Study

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Behçet's syndrome (BS) commonly presents with mucocutaneous and joint disorders.
• Effective treatments for joint lesions in BS remain a significant clinical need.
• Minor forms of BS often involve clustered mucocutaneous and joint symptoms.

Purpose of the Study:

• To evaluate the long-term safety and effectiveness of apremilast for refractory joint and mucocutaneous manifestations of Behçet's syndrome.
• To assess apremilast's efficacy in patients resistant to colchicine and disease-modifying antirheumatic drugs (DMARDs).

Main Methods:

• A French nationwide multicenter study involved 50 BS patients with active, treatment-resistant joint and/or mucocutaneous manifestations.
• Patients received apremilast 30 mg twice daily.
• The primary endpoint was complete response (CR) of articular symptoms at 6 months, defined as resolution of inflammatory arthralgia and arthritis with a zero joint count.

Main Results:

• Complete response of joint disease at 6 months was achieved by 65% of patients; 17% were partial responders.
• High CR rates were observed for oral ulcers (73%), genital ulcers (94%), and pseudofolliculitis (71%).
• The overall response rate at 6 months was 74%, with a significant decrease in the Behçet's syndrome activity score (p < 0.0001).

Conclusions:

• Apremilast demonstrates effectiveness in treating Behçet's syndrome-related articular disease refractory to standard treatments.
• Discontinuation rates due to adverse events were notably higher than those reported in previous clinical trials.
• Gastrointestinal and neurological adverse events were the most common reasons for apremilast withdrawal.