Effects of Longterm Treatment with Bosentan and Iloprost on Nailfold Absolute Capillary Number, Fingertip Blood Perfusion, and Clinical Status in Systemic Sclerosis

Abstract

Objective.To quantify in patients with systemic sclerosis (SSc) the absolute nailfold capillary number/mm (the absolute number of capillaries, observable in the first row, in 1 mm per field) and fingertip blood perfusion (FBP) during longterm therapy with the endothelin receptor antagonist bosentan (BOSE) and the synthetic analog of prostacyclin PGI2 iloprost (ILO) by multiple diagnostic tools. Observed values were correlated with clinical outcomes.Methods.Thirty patients with SSc already receiving intravenous ILO (80 μg/day) for 5 continuous days (every 3 mos) were recruited in the clinic. 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 (DU). The followup period was 4 years (T0–T4). Every year the following were evaluated: absolute nailfold capillary number/mm by nailfold videocapillaroscopy, FBP by laser Doppler flowmetry, DU incidence, DLCO, systolic pulmonary arterial pressure (sPAP), renal arterial resistive index, and other biomarkers. From T2 to T4, laser speckled contrast analysis was added. Nonparametric tests were used for statistical analysis.Results.Limited to the ILO + BOSE group, absolute capillary number/mm and FBP showed a progressive increase independently from other variables. In addition, during followup there was a significant reduction (80%) in the incidence of new DU, whereas DLCO and sPAP did not worsen.Conclusion.The study shows in patients with SSc with up to 4 years of combined therapy a progressive significant recovery in structure and function of microvasculature linked to improved clinical outcomes, independent of disease severity.

Automated assessment of absolute nailfold capillary number on videocapillaroscopic images: Proof of principle and validation in systemic sclerosis

“…Recent evidences, in large SSc cohorts, confirmed that the number of capillaries, as detected by NVC, is the most reliable capillaroscopic parameter for prediction of disease progression and detection of effects of therapies . Additionally, capillary density was used for the construction of several scoring systems such as the CSURI, the MES, and the simple day‐to‐day risk index .…”

“…N = number. very good reliability of the software was confirmed, according to results of single measures ICC, in the difficult SSc image subsets, but also in the easier images obtained from healthy subjects and PRP subject subsets, used as controls.Recent evidences, in large SSc cohorts, confirmed that the number of capillaries, as detected by NVC, is the most reliable capillaroscopic parameter for prediction of disease progression and detection of effects of therapies 4,5,42,43. Additionally, capillary density was used for the construction of several scoring systems such as the CSURI, the MES, and the simple day-to-day risk index 4,28,44.…”

The Impact of Nailfold Capillaroscopy in the Approach of Microcirculation

“…Following disease progression, dynamic transition of microvascular abnormalities through different NFC patterns can be found in up to 50% of SSc patients [18,26]. On the other hand, capillaroscopy patterns can improve after up to 4 years of combined treatment, revealing a progressive significant recovery in structure and function of microcirculation, associated to ameliorated outcomes, independently of disease severity [27][28][29].…”

Nailfold capillaroscopy in rheumatology: ready for the daily use but with care in terminology

“…In addition, the NVC patterns have been found to change in patients with SSc with up to 4 years of combined therapy, in particular showing a progressive significant recovery in structure as well as function of microvasculature, linked to improved clinical outcomes, independent of disease severity [20][21][22].…”