title: Longterm treatment with endothelin receptor antagonist bosentan and iloprost improves fingertip blood perfusion in systemic sclerosis.
abstract: OBJECTIVE
  To evaluate the longterm effects of endothelin-1 (ET-1) antagonism on peripheral blood perfusion (PBP) in patients with systemic sclerosis (SSc).
  
  
  METHODS
  Twenty-six patients with SSc already receiving cyclic intravenous iloprost (ILO) for severe Raynaud phenomenon were enrolled. Thirteen patients continued the treatment for a further 3 years (ILO group) and 13 patients, because of the appearance of digital ulcers, received in addition bosentan (BOS; 125 mg twice/day) for 3 years (ILO + BOS group). Both PBP at fingertips and nailfold microangiopathy were evaluated yearly by laser Doppler flowmetry and nailfold videocapillaroscopy, respectively.
  
  
  RESULTS
  A progressive significant increase of PBP was observed in the ILO + BOS group during the 3 followup years (p = 0.0007, p = 0.0002, p = 0.01, respectively). In contrast, an insignificant progressive decrease of PBP was observed in the ILO group. Difference of perfusion between the PBP evaluations at basal temperature and at 36 °C (to test capillary dilation capacity), was found progressively decreased during the 3-year followup only in the ILO group (p = 0.05, p = 0.26, p = 0.09, respectively). A progressive increase of nailfold capillary number was observed only in the ILO + BOS group after 2 and 3 years of followup (p = 0.05).
  
  
  CONCLUSIONS
  Longterm treatment of SSc patients with ET-1 antagonism, in combination with ILO, seems to increase fingertip blood perfusion, as well as both capillary dilation capacity and number.
authors.affiliation: From the Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Cutolo
authors.forename: Barbara
authors.initials: B
authors.lastname: Ruaro
authors.forename: Carmen
authors.initials: C
authors.lastname: Pizzorni
authors.forename: Francesca
authors.initials: F
authors.lastname: Ravera
authors.forename: Vanessa
authors.initials: V
authors.lastname: Smith
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Zampogna
authors.forename: Sabrina
authors.initials: S
authors.lastname: Paolino
authors.forename: Bruno
authors.initials: B
authors.lastname: Seriolo
authors.forename: Marco
authors.initials: M
authors.lastname: Cimmino
authors.forename: Alberto
authors.initials: A
authors.lastname: Sulli
mesh_terms: D000368:Aged; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use*; D005260:Female; D005385:Fingers / Q000098:blood supply*; D006801:Humans; D016285:Iloprost / Q000627:therapeutic use*; D017078:Laser-Doppler Flowmetry; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D011446:Prospective Studies; D012039:Regional Blood Flow / Q000187:drug effects*; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D013449:Sulfonamides / Q000627:therapeutic use*; D013997:Time Factors; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D014665:Vasodilator Agents; D016285:Iloprost; D000077300:Bosentan
keywords: ENDOTHELIN-1 ANTAGONIST; ILOPROST; LASER DOPPLER FLOWMETRY; NAILFOLD VIDEOCAPILLAROSCOPY; RAYNAUD PHENOMENON; SYSTEMIC SCLEROSIS