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Prediction and primary prevention of major vascular complications in systemic sclerosis

Published:February 05, 2021DOI:https://doi.org/10.1016/j.ejim.2021.01.022

      Highlights

      • presence of dyspnoea and arthritis are confirmed risk factors for development of major vascular complications in systemic sclerosis.
      • vasoactive and vasodilating drugs may not prevent the onset of major vascular complications in scleroderma patients.
      • while prostanoids and angiotensin receptors blockers may delay the onset of major vascular complications, angiotensin converting enzyme inhibitors seem to reduce the time to their development.

      Abstract

      Objective

      In Systemic Sclerosis (SSc), vasculopathy is the background of major vascular complications (MVCs), like digital ulcers (DUs), pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC). We aimed to identify the predictors and to test the primary preventive effect of vasoactive/vasodilating drugs (VVD) for the development of MVCs in SSc MVCs-naïve patients.

      Methods

      patients fulfilling the ACR/EULAR 2013 classification criteria for SSc without history of MVCs were eligible. Data about clinical manifestations, laboratory and instrumental assessments and treatments were retrospectively collected at baseline and latest available follow-up.

      Results

      134 SSc patients were enrolled (mean age 56.5 years ± 14.2, females 88.1%, limited subset 61.9%, ACA positivity 60.4%). In a mean of 43 ± 19 months of follow-up 12 (9.0%) patients developed at least 1 MVC (10 DU, 2 PAH and 1 SRC). Dyspnoea and arthritis at baseline were independent predictors for MVCs development (p = 0.012, and p = 0.002 respectively). No primary preventive effect of VVD on MVCs development was found. However, sildenafil reduced the renal resistive index increase (p = 0.042) and alprostadil slowed the DLco decline (p = 0.029). Both iloprost and angiotensin-receptor blockers (ARBs) delayed MVCs development, while angiotensin converting enzyme inhibitors (ACEi) determined an earlier onset of such MCVs.

      Conclusions

      in SSc patients, our data confirm the role of arthritis and dyspnea as independent predictors of major vascular complications, in particular in MVCs-naïve patients. Prostanoids, sildenafil and ARBs, even in absence of a primary preventive action, might help in slowing disease progression and postponing the onset of MVCs.

      Keywords

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