Background/Purpose:  In systemic sclerosis (SSc), microvascular damage progresses from capillary dilation to capillary loss and reactive angiogenesis, as detectable by nailfold videocapillaroscopy (NVC) [1]. The process is systemic and determines multiple clinical manifestations, from the early appearance of Raynaud’s phenomenon, through formation of digital ulcers (DUs), until systemic organ involvement [2,3]. Objective of the study was to quantify, in SSc patients, absolute nailfold capillary number/mm and fingertip blood perfusion (FBP) during long-term treatment with the endothelin receptor antagonist (ERA) bosentan (BOSE) and the synthetic analogue of prostacyclin PGI2 iloprost (ILO) by multiple diagnostic tools. Observed values were correlated with clinical outcomes.

Methods: Thirty SSc patients, already receiving intravenous ILO (80 μg/day), for 5 continuous days (every 3 months) were recruited. Fifteen patients continued such treatment (ILO group), while in 15 patients BOSE (125 mg twice/day) was added (ILO+BOSE group), because of the onset of pulmonary arterial hypertension or digital ulcers (DUs). The follow-up was of 4 years (T0-T4). The following diagnostic tests were performed on a yearly basis: absolute nailfold capillary number/mm count by nailfold videocapillaroscopy (NVC), FBP by laser Doppler flowmetry (LDF) evaluation, DUs incidence, diffusing capacity of the lung for carbon monoxide (DLCO), systolic pulmonary arterial pressure (sPAP), renal arterial resistive index (RI) and other biomarkers analysis. From T2 to T4 laser speckled contrast analysis (LASCA) was added. Non-parametric tests were used for statistical analysis.

Results: Only in the ILO+BOSE group, multivariate analysis showed that absolute capillary number/mm and FBP had a progressive increase (p= 0.01 and p<0.0001, respectively), independently from other clinical variables. In the same group LASCA analysis of fingertips showed a significant improvement (p= 0.045). In addition, during follow up there was a significant reduction (80%) in the incidence of new DUs (p= 0.002), whereas DLCO and sPAP did not worse. No relevant side effects were observed.

Conclusion:  The study shows in SSc patients up to four years of combined therapy, a progressive significant recovery in structure and function of microvasculature, together with improved clinical outcomes, independently from disease severity. 1] Cutolo M et al. Nat Rev Rheumatol 2010;6:578-87; [2] Berezne A et al. Arthritis Care Res 2011;63:277-85; [3] Wigley FM. Clin Rev Allergy Immunol 2009;36:150-75.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/four-year-effects-of-combined-bosentan-and-iloprost-treatment-on-nailfold-absolute-capillary-number-fingertip-blood-perfusion-and-clinical-status-in-systemic-sclerosis-patients/