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Updated: Jun 14, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Effect of bosentan on skin fibrosis in patients with systemic sclerosis: a prospective, open-label, non-comparative
Annegret Kuhn1, Merle Haust, Vincent Ruland
1Department of Dermatology, University of Münster, Münster, Germany. kuhnan@uni-muenster.de
Objectives:
To assess the effect of the ET-receptor antagonist bosentan on skin fibrosis and functionality in patients with SSc.
Methods:
In this prospective, open-label, non-comparative trial, a total of 10 patients with SSc received 62.5 mg of bosentan twice daily for 4 weeks and then 125 mg twice daily for 20 weeks. The primary endpoint was skin thickening as measured by the modified Rodnan skin score (mRSS). Further assessments included 20 MHz ultrasound, examination of digital ulcers (DUs) and evaluation of hand function by examining patients' fist closure. Furthermore, patients with SSc used the UK SSc Functional Score (UKFS), the modified scleroderma HAQ (SHAQ) and its visual analogue scale (VAS) to rate their disability related to specific organ systems.
Results:
The mean change from baseline mRSS (the primary endpoint) was 6.4 at Week 24 of bosentan treatment, which was statistically significant (P < 0.001). Patients with both diffuse and limited SSc exhibited a statistically significant mean difference in the mRSS. Moreover, there was a significant healing of DUs noted between baseline and at Week 24 of bosentan treatment (P < 0.001); however, the 20 MHz ultrasound and the fist closure evaluation revealed no significant differences. There were also no statistically significant changes between baseline and Week 24 in the UKFS, the modified SHAQ and its VAS.
Conclusion:
In addition to the well-known effect of bosentan in prevention of DUs, the results of this study demonstrate that bosentan may also be effective at reducing skin fibrosis in patients with SSc.
