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AB0583 Long-Term Integrated Treatment with Bosentan and Iloprost Increases, Independently from Other Clinical Characteristics, The Absolute Number of Capillaries in Systemic Sclerosis Patients: A Nailfold Capillaroscopic Analysis
  1. A.C. Trombetta1,
  2. A. Sulli1,
  3. C. Pizzorni1,
  4. S. Paolino1,
  5. E. Alessandri1,
  6. V. Smith2,
  7. B. Ruaro1,
  8. M. Cutolo1
  1. 1Research Laboratory and Academic Division of Clinical Rheumatology Department of Internal Medicine, University of Genova, Genova, Italy
  2. 2Department of Rheumatology, Ghent University Hospital, Faculty of internal medicine, Gent University, Gent, Belgium


Background Iloprost (ILO) and bosentan (BOSE) integrated therapy was demonstrated to improve microvascular structure in systemic sclerosis (SSc) patients, in two long-term follow up studies [1].

Objectives Since the alterations in capillary number seem to predict the incidence of clinical SSc complications [2], present study aimed to quantify, by nailfold videocapillaroscopy (NVC), long-term effects of BOSE treatment on nailfold capillary absolute number and their possible correlation with clinical parameters of the disease.

Methods Thirty SSc patients entered the study, with a follow up of for 4 years. Fifteen patients were treated with ILO mono-therapy (80 μg/day, for 5 continuous days, every 3 months), while other 15 patients, because of the onset of systolic pulmonary hypertension (sPAP) or digital ulcers (DUs) were treated with BOSE (125 mg twice/day) and ILO integrated therapy (ILO+BOSE). Nailfold capillaries absolute number, DU incidence, diffusing capacity of the lung for carbon monoxide (DLCO), sPAP and renal artery resistive index (RI) were evaluated yearly. Friedman test, Cochran's q test, Marginal homogeneity and mixed modelling were used to study variables with repeated measures. A p value lower then 0.05 was considered significant.

Results In the ILO+BOSE group, capillary absolute number was found to have a significant increase (p=0.01) from T0: 6.8±1.3 to T4: 7.8±1.5 (14.7%), whereas in the ILO group, a non-significant decrease in capillary number from T0: 7.18±0.5 to T4: 6.5±1.1 (9.4%) was found. According to multivariate analysis, the most significant associations, other then BOSE treatment, were observed between capillary number increase and baseline capillaroscopic “Early” pattern (p<0.0001), lcSSc subtype (p=0.001), while it was independent from other clinical characteristics. In the ILO+BOSE group there was a significant reduction in the annual incidence of new DUs from T0 (10/15 patients) to T4 (2/15 patients) (80%; p=0.0042) and no further negative significant changes were observed in DLCO and sPAP. On the contrary, in the ILO group, DLCO was significantly reduced (8.1%, p=0.039) and sPAP increased (15%, p=0.04) during the follow up. RI did not change significantly in both groups.

Conclusions Capillary absolute number count seems a reliable parameter to evaluate long-term effects of BOSE on microvasculature in SSc. A significant reduction in the annual incidence of new DUs and stabilization of lung function was observed and appeared to be associated to the increased number of capillaries during a four-years integrated treatment with BOSE and ILO.

  1. Cutolo M et al. 2013;40:40–5.

  2. Smith V et al. 2011;70:180–3.

Disclosure of Interest None declared

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