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Rheumatology International

, Volume 34, Issue 2, pp 221–225 | Cite as

Improvement of plasma endothelin-1 and nitric oxide in patients with systemic sclerosis by bosentan therapy

  • Shin-ya KawashiriEmail author
  • Yukitaka Ueki
  • Kaoru Terada
  • Satoshi Yamasaki
  • Kiyoshi Aoyagi
  • Atsushi Kawakami
Original Article

Abstract

The aim of this study was to evaluate the effects of bosentan on plasma endothelin-1 (ET-1) and nitric oxide (NO) as pulmonary hypertension (PH)-associated biochemical markers in patients with systemic sclerosis (SSc). Twenty-four SSc patients receiving bosentan for 24 weeks were registered in this prospective observational study. Ten patients were complicated with clinically suspected PH. Plasma levels of ET-1 and NO were assessed at baseline and after 24 weeks of treatment in SSc patients and in 15 healthy controls. Plasma levels of ET-1 and NO at baseline were significantly higher in SSc patients than in healthy controls (p < 0.000), and they were also significantly higher in SSc patients with PH than in those without PH (p < 0.01). Plasma ET-1 levels were significantly decreased after 24 weeks of bosentan therapy (p < 0.0001), and ET-1 levels of SSc patients with PH decreased to a level comparable to that in patients without PH. In the 10 SSc patients with PH, changes in plasma ET-1 levels during the 24 weeks of the study were significantly larger in the 5 patients whose functional class (FC) improved than in the 5 patients whose FC was unchanged (p < 0.05). Plasma NO levels were also slightly decreased in SSc patients after 24 weeks of bosentan therapy. Plasma ET-1 levels could reflect the presence and severity of PH in SSc patients. Additionally, changes in plasma ET-1 levels may indicate the response to bosentan therapy in SSc patients with PH.

Keywords

Bosentan Systemic sclerosis Pulmonary hypertension Endothelin-1 Nitric oxide 

Abbreviations

cGMP

Cyclic guanosine monophosphate

ELISA

Enzyme-linked immunosorbent assay

ET

Endothelin

FC

Functional class

NO

Nitric oxide

PAH

Pulmonary arterial hypertension

PAP

Pulmonary artery pressure

PH

Pulmonary hypertension

SSc

Systemic sclerosis

WHO

World Health Organization

6MWD

6-minute walk distance

Notes

Conflict of interest

None.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Shin-ya Kawashiri
    • 1
    • 2
    Email author
  • Yukitaka Ueki
    • 3
  • Kaoru Terada
    • 3
  • Satoshi Yamasaki
    • 2
  • Kiyoshi Aoyagi
    • 1
  • Atsushi Kawakami
    • 2
  1. 1.Department of Public Health, Graduate School of Biomedical SciencesNagasaki UniversityNagasakiJapan
  2. 2.Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical SciencesNagasaki UniversityNagasakiJapan
  3. 3.Center for Rheumatic DiseaseSasebo Chuo HospitalSaseboJapan

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