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Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Endothelin-1 levels in scleroderma patients: a pilot study.

Emanuele Cozzani1, Sanja Javor, Erika Laborai

  • 1DISSAL, Section of Dermatology, IRCCS University Hospital San Martino-IST, 16132 Genoa, Italy.

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PubMed
Summary
This summary is machine-generated.

Serum endothelin-1 (ET-1) levels are elevated in systemic sclerosis (SSc) and correlate with disease severity. However, ET-1 levels do not predict digital ulcer development, though bosentan therapy reduces ET-1 in patients with digital ulcers.

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Area of Science:

  • Vascular Biology
  • Rheumatology
  • Pharmacology

Background:

  • Endothelin-1 (ET-1) is a potent vasoconstrictor involved in vascular cell proliferation, fibrosis, and inflammation via ET-A and ET-B receptors.
  • Systemic sclerosis (SSc) is associated with vascular complications like digital ulcers (DUs).

Purpose of the Study:

  • To investigate the correlation between serum ET-1 levels and the presence/development of DUs in SSc patients.
  • To assess the impact of ET-1 antagonist therapy (bosentan) on ET-1 levels in SSc patients with DUs.

Main Methods:

  • Retrospective study analyzing serum ET-1 levels in 18 SSc patients with and without DUs.
  • Correlation analysis of ET-1 levels with SSc severity and DU development.
  • Evaluation of ET-1 levels before and during bosentan therapy.

Main Results:

  • All SSc patients exhibited higher-than-normal serum ET-1 levels, correlating with disease severity.
  • Patients without DUs had higher ET-1 levels, which did not correlate with new DU development.
  • Bosentan therapy in patients with DUs led to a reduction in serum ET-1 levels.

Conclusions:

  • Serum ET-1 levels do not correlate with the development of digital ulcers in SSc patients.
  • Bosentan is effective in treating and preventing DUs, evidenced by the reduction in ET-1 levels.
  • ET-A receptor inhibition by bosentan may enhance ET-B receptor-mediated ET-1 degradation.