RT Journal Article SR Electronic T1 Longterm Treatment with Endothelin Receptor Antagonist Bosentan and Iloprost Improves Fingertip Blood Perfusion in Systemic Sclerosis JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP jrheum.131284 DO 10.3899/jrheum.131284 A1 Maurizio Cutolo A1 Barbara Ruaro A1 Carmen Pizzorni A1 Francesca Ravera A1 Vanessa Smith A1 Giuseppe Zampogna A1 Sabrina Paolino A1 Bruno Seriolo A1 Marco Cimmino A1 Alberto Sulli YR 2014 UL http://www.jrheum.org/content/early/2014/03/26/jrheum.131284.abstract AB 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). Conclusion 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.