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Updated: May 30, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Effects of bosentan on nondigital ulcers in patients with systemic sclerosis
T Taniguchi1, Y Asano, M Hatano
1Departments of Dermatology and Cardiovascular Medicine, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Background:
Bosentan is an oral dual endothelin receptor antagonist, which has been shown to be efficacious for preventing new digital ulcers in patients with systemic sclerosis (SSc) in two high-quality randomized controlled trials. However, its efficacy for nondigital ulcers in SSc remains unknown.
Objectives:
To evaluate the efficacy of bosentan on nondigital ulcers in patients with SSc.
Methods:
Bosentan was administered to five patients with SSc with pulmonary arterial hypertension, who also had nondigital ulcers refractory to conventional treatments. The efficacy of bosentan on nondigital ulcers and its association with clinical features of ulcers were analysed.
Results:
The nondigital ulcers refractory to conventional treatments were significantly improved by the administration of bosentan in cases surrounded with severe cyanosis. In contrast, nondigital ulcers without cyanosis were still refractory to bosentan therapy.
Conclusions:
Bosentan may be efficacious for accelerating the healing of nondigital ulcers with severe cyanosis, suggesting that nondigital ulcers caused by severely impaired peripheral circulation are highly responsive to this treatment.
Insights
Bosentan effectively treats severe cyanotic nondigital ulcers in systemic sclerosis (SSc) patients. This dual endothelin receptor antagonist shows promise for ulcers caused by poor circulation.
Area of Science:
- Vascular Medicine
- Dermatology
- Rheumatology
Background:
- Systemic sclerosis (SSc) patients often develop digital and nondigital ulcers.
- Bosentan, a dual endothelin receptor antagonist, is approved for preventing digital ulcers in SSc.
- Efficacy of bosentan for SSc-related nondigital ulcers is not well-established.
Observation:
- This study investigated bosentan's efficacy in five SSc patients with pulmonary arterial hypertension and refractory nondigital ulcers.
- Ulcer characteristics, particularly cyanosis, were analyzed in relation to treatment response.
Findings:
- Bosentan significantly improved nondigital ulcers associated with severe cyanosis.
- Ulcers without severe cyanosis showed limited response to bosentan therapy.
Implications:
- Bosentan may accelerate healing of nondigital ulcers in SSc patients with severe cyanosis.
- This suggests that impaired peripheral circulation is a key factor in ulcer responsiveness to bosentan.
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