Cutolo Maurizio, Ruaro Barbara, Pizzorni Carmen, Ravera Francesca, Smith Vanessa, Zampogna Giuseppe, Paolino Sabrina, Seriolo Bruno, Cimmino Marco, Sulli Alberto
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.
J Rheumatol. 2014 May;41(5):881-6. doi: 10.3899/jrheum.131284. Epub 2014 Apr 1.
To evaluate the longterm effects of endothelin-1 (ET-1) antagonism on peripheral blood perfusion (PBP) in patients with systemic sclerosis (SSc).
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.
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).
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.
评估内皮素-1（ET-1）拮抗剂对系统性硬化症（SSc）患者外周血灌注（PBP）的长期影响。
招募26例已接受环磷酰胺静脉注射伊洛前列素（ILO）治疗严重雷诺现象的SSc患者。13例患者继续治疗3年（ILO组），13例患者因出现指端溃疡，加用波生坦（BOS；125mg，每日2次）治疗3年（ILO + BOS组）。分别通过激光多普勒血流仪和甲襞视频毛细血管镜每年评估指尖PBP和甲襞微血管病变。
在3年随访期间，ILO + BOS组的PBP显著逐步增加（分别为p = 0.0007，p = 0.0002，p = 0.01）。相比之下，ILO组的PBP出现不显著的逐步下降。仅在ILO组中，3年随访期间发现基础温度和36°C时PBP评估之间的灌注差异（用于测试毛细血管扩张能力）逐渐降低（分别为p = 0.05，p = 0.26，p = 0.09）。仅在ILO + BOS组随访2年和3年后观察到甲襞毛细血管数量逐渐增加（p = 0.05）。
ET-1拮抗剂联合ILO对SSc患者进行长期治疗似乎可增加指尖血灌注以及毛细血管扩张能力和数量。
