Efficacy and safety of apremilast for Behçet's syndrome: a real-life single-centre Italian experience

Rheumatology (Oxford). 2020 Jan 1;59(1):171-175. doi: 10.1093/rheumatology/kez267.

Abstract

Objectives: To evaluate the efficacy and safety of apremilast in treating oral ulcers (OUs), the cardinal and high-disabling feature of Behçet's disease (BD).

Methods: Twelve consecutive patients affected by BD with recurrent/relapsing OUs resistant and/or intolerant to conventional therapy were enrolled and prospectively followed. The primary endpoint was the number of OUs at week 12. Secondary endpoints were modification from baseline to week 12 in Behçet's Syndrome Activity Score (BSAS), Behçet's Disease Current Activity Form (BDCAF) score, Behçet's Disease Quality of Life (BDQOL) scale and pain of OUs, as measured by a visual analogue scale (VAS). All adverse events (AEs) were recorded during follow-up. Non-parametric tests (Wilcoxon rank test) were used and a P-value <0.05 was considered statistically significant.

Results: After 12 weeks of apremilast, there was a significant reduction in the number of OUs [0.58 (s.d. 0.67) vs 3.33 (s.d. 1.45) at baseline, P = 0.02] that was paralleled by improvement in disease activity: BSAS was 16.8 (s.d. 9.1) [from 45.9 (s.d. 19.6) at baseline] (P = 0.02), BDCAF score was 0.72 (s.d. 0.65) [vs 2.45 (s.d. 1.0) at baseline] (P = 0.04) and the VAS score for pain decreased to 23.3 (s.d. 13.7) [vs 67.9 (s.d. 17.2) at baseline] (P = 0.02). Consistently, an improvement of BDQOL was assessed (P = 0.02). Clinical improvement led to complete steroid discontinuation in six patients and a tapering of the prednisone dose in two patients (P = 0.016). Colchicine was discontinued in six of nine patients (P = 0.031). AEs related to apremilast occurred in four patients (mainly due to gastrointestinal AEs), leading to drug discontinuation in all of them.

Conclusion: Our preliminary real-world data support the use of apremilast as an effective therapeutic strategy against BD-related recurrent OUs resistant or intolerant to first-line therapy.

Keywords: Behçet’s disease; apremilast; oral ulcers.

MeSH terms

  • Adult
  • Anti-Inflammatory Agents, Non-Steroidal / administration & dosage
  • Behcet Syndrome / complications
  • Behcet Syndrome / drug therapy*
  • Behcet Syndrome / epidemiology
  • Dose-Response Relationship, Drug
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Italy / epidemiology
  • Male
  • Mouth Mucosa / pathology*
  • Oral Ulcer / drug therapy
  • Oral Ulcer / epidemiology
  • Oral Ulcer / etiology
  • Prospective Studies
  • Quality of Life*
  • Recurrence
  • Thalidomide / administration & dosage
  • Thalidomide / analogs & derivatives*
  • Time Factors
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Thalidomide
  • apremilast