Abstract

Long-term Efficacy snd Safety of Bosentan in Patients with Digit al Ulcers Related to Systemic Sclerosis

Objective: Two pivotal studies, RAPIDS-1 and RAPIDS-2 (RAndomized, double-blind, Placebo-controlled study with bosentan on healing and prevention of Ischemic Digital ulcers in patients with systemic Sclerosis) revealed that Bosentan reduces the development of new Digital Ulcers (DUs) in patients with Systemic Sclerosis (SSc). However, data regarding the long-term use of this dual endothelin antagonist receptor in the treatment of DUs is scarce. Methods: We conducted a prospective observational case-control study, between 2014 and 2020 that enrolled 80 SSc patients with at least one active DU at baseline compatible with a vascular etiology or recurrent DUs within the previous 3 months. DUs number, patients’ subjective perception of DUs’ pain and/or Raynaud’s phenomenon, nail fold video-capillaroscopy, and Health Assessment Questionnaire (HAQ) were reassessed every 6 months, for up to 60 months after treatment initiation. Results: At week 24, bosentan therapy was associated with a significant reduction in the number of DUs (p<0.001) and significant improvement of quality of life (p<0.001), patients’ subjective perception of DUs’ pain (p<0.001) and Raynaud’s phenomenon (p<0.001) compared to baseline and benefits were maintained up to month 60. Long-term use of bosentan also improved the Microangiopathy Evolution Score (MES) and the difference was statistically significant between bosentan-treated and the control group (p=0.005). Accelerated development of new DUs was described 6 months after temporarily stopping bosentan. Following the re-initiation of treatment, the mean number of DUs rapidly decreased. Conclusion: Bosentan has long-term efficacy in DUs prevention in SSc patients, a tolerable safety profile, and might improve microvascular remodeling.

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