Improvement with time of vascular outcomes in systemic sclerosis: a systematic review and meta-analysis study.

Objectives: Vascular disease in SSc is associated with significant morbidity and mortality. Preliminary data may lead to the suggestion of a modifiable unified-vascular endophenotype. Our aim was to determine whether the prevalence, mortality and severity of SSc-vascular disease have changed over time.
Methods: We performed a systematic review and meta-analysis of the literature in PubMed 1950-2019 related to SSc-digital ulcers (DUs), pulmonary artery hypertension (PAH) and scleroderma renal crisis (SRC). We included full-text articles and extracted study characteristics and assessed risk of bias/quality. We examined the prevalence, mortality and surrogate measures of SSc-associated vascular disease severity.
Results: We included 55 studies in our meta-analysis. The pooled prevalence of DUs (41.0%), PAH (9.5%) and SRC (4.9%) remained largely stable over time. There was significant improvement in PAH 1-year (P = 0.001) and SRC mortality (P < 0.001), but not PAH 3-year (P = 0.312) or 5-year (P = 0.686) mortality. The prevalence of DU healing did not significantly change (P = 0.265). There was a trend (all P = ∼0.1) towards improvement in PAH surrogates: mean pulmonary artery pressure, pulmonary vascular resistance and right atrial pressure. For SRC, there was evidence that the overall frequency of dialysis (66.7%, P = 0.297) and permanent dialysis (35.4%, P = 0.036) increased over time.
Conclusion: Despite the heterogeneity and scarcity of the disease, there have been major improvements obtained in the various vascular complications in SSc leading to benefit in survival. This is supported by a trend towards improvement in several surrogate markers and demonstrates that progress in vascular management translates into major patient benefit.
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