Bosentan is Effective Against Digital Ulcerations and Hyperkeratosis in Systemic Sclerosis

Kurgyis, Zsuzsanna; Varga, Rita; Sick, Isabell; Lang, Martin Ulrich; Ruzicka, Thomas; Sárdy, Miklós

doi:10.2340/00015555-1138

Cited by 4 publications

(3 citation statements)

References 13 publications

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“…Moreover, patients numbers 1 and 5 treated with bosentan showed a complete clinical improvement of digital ulcers and remission of pain in about 3 months correlated with a reduction of ET-1 serum levels (15% and 23%, resp.) and none developed new DUs in accordance with data of the literature [5]. On the contrary DUs patients treated with calcium antagonist (patients: numbers 3, 8, 10, 12, and 13) had a slightly longer clinical remission.…”

Section: Resultssupporting

confidence: 87%

Endothelin-1 Levels in Scleroderma Patients: A Pilot Study

et al. 2013

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Endothelin-1 (ET-1) is a potent endogenous vasoconstrictor, which mediates vascular wall cells proliferation, fibrosis, and inflammation through two types of ET-1 receptors (ET-A and ET-B). In our retrospective study the serum levels of ET-1 in 18 systemic sclerosis (SSc) patients with and without digital ulcers (DUs) were assessed to observe possible correlation between the levels of ET-1, the evolution of SSc, and the therapy with an ET-1 antagonist (bosentan). In all our patients, the levels of ET-1 were found higher than normal range and correlate with the severity of the disease. Furthermore we also observed that in patients without DUs the levels of ET-1 were higher and did not correlate with new DUs development. In conclusion, the levels of ET-1 in our studied patients do not correlate with the possible development of DUs. The reduction of ET-1 levels in DUs patients in therapy with bosentan confirms the efficacy of this molecule both for treatment and prevention of digital ulcers. The inhibition of ET-A receptor by its antagonist may activate the opposite ET-B receptors, with well-known function ET-1 degradation and reducing of ET-1 serum level as confirmed in our pilot study.

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Section: Resultssupporting

confidence: 87%

Endothelin-1 Levels in Scleroderma Patients: A Pilot Study

et al. 2013

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“…8 However, recent articles have reported bosentan’s effectiveness as digital ulcers treatment in systemic sclerosis. 9,10 In our case, it is noteworthy that there was a clear-cut improvement after bosentan was started. We, for the first time, have associated ECP and bosentan in EF treatment.…”

mentioning

confidence: 51%

Bosentan and Extracorporeal Photochemotherapy in Eosinophilic Fasciitis

Pileri

Odorici

Giudice

et al. 2014

The International Journal of Lower Extremity Wounds

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“…However, several additional publications suggest that bosentan may provide an effective and swift response in the treatment of digital ulcers [13–19], as observed in the case herein presented. Macitentan also provided rapid (less than 3 months) and complete healing while maintaining a normal hepatic function and without observed DU recurrence after 6 months, arguing in favor of its use.…”

Section: Discussionmentioning

confidence: 64%

Swift and Complete Healing of Digital Ulcers after Macitentan Treatment

Serret

2016

Case Reports in Rheumatology

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Digital ulcers are a burdensome and painful condition with sparse options of treatment. We report the case of a 78-year-old female patient with limited cutaneous systemic sclerosis that sequentially developed digital ulcers. After the appearance of digital ulcers in the soles of her feet she was successfully treated with bosentan. The report of two new digital ulcers in her hands 9 months later alongside with elevated transaminase levels led to a switch to macitentan treatment. A swift and complete healing of both digital ulcers was observed after 3 months, with the restoration of normal biochemical values.

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Bosentan is Effective Against Digital Ulcerations and Hyperkeratosis in Systemic Sclerosis

Cited by 4 publications

References 13 publications

Endothelin-1 Levels in Scleroderma Patients: A Pilot Study

Endothelin-1 Levels in Scleroderma Patients: A Pilot Study

Bosentan and Extracorporeal Photochemotherapy in Eosinophilic Fasciitis

Swift and Complete Healing of Digital Ulcers after Macitentan Treatment

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