Abstract

BackgroundIntravenous iloprost is currently recommended in the treatment of Raynaud’s phenomenon (RP) refractory to oral therapy and of digital ulcers (DUs) related to systemic sclerosis (SSc). In real-life practice there is a huge heterogeneity about the Iloprost regimens used, because of a lack of specific recommendations. Coronavirus pandemic resulted in an unexpected emergency leading to sudden and synchronous withdrawal of programmed iloprost infusions for most SSc patients in March 2020, in order to limit virus dissemination. At the same time, lockdown forced people to stay at home, thus reducing the exposure to coldness. Both these unavoidable circumstances were close to an experimental condition, clearly non-replicable in routine conditions.ObjectivesThe aim of the survey was to evaluate the consequences related to a sudden and simultaneous iloprost discontinuation in a cohort of SSc patients.MethodsA telephone survey was carried out on SSc patients that interrupted Iloprost infusion at our centre. They were specifically asked to compare acral vascular symptoms just before Iloprost withdrawal, in February and just after the missed infusion in March (0-10 scale). Demographic and disease characteristics, severity and frequency of RP, new DUs onset or aggravation of those pre-existing were reported for each patient. Last available capillaroscopic images were also evaluated to assess the pattern.ResultsThe analysis included 50 patients. After iloprost withdrawal, 11 patients reported a RP worsening because of enhanced intensity (4.6±2.1 vs 5.2±2.3, p=0.007). Only 8 patients of them also complained of an increased frequency (4.5±2.3 vs 5.0±2.4, p=0.07). None of the patients experienced digital ulcers for the first time during quarantine. Among the 27 patients with a history of digital ulcers, 9 reported worsening and 7 recurrences of DUs. Overall, 17 patients (34.0%) complained of a worsening of SSc vascular acral manifestations, namely RP or DUs. Disease and general features did not statistically correlate with worsening of RP except for reduced capillary density. Of note, each unit increase of capillary density corresponds to an average 44% decrease in the odds of RP worsening (OR 0.56, CI 95% 0.36-0.97, p=0.037). History of DUs - either active or former at the time of iloprost discontinuation - was the only clinical predictor of worsening of DU severity. As for RP worsening, the worsening of DU was associated with a lower capillary density.ConclusionLow capillary density can predict a worsening of both RP and DUs within a month after iloprost discontinuation in SSc patients. Further studies are needed to assess whether the capillaroscopy should be used to personalize iloprost regimen in SSc patients.Disclosure of InterestsNone declared

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