Effectiveness of oral anticoagulants in precapillary pulmonary hypertension associated with systemic sclerosis: a EUSTAR cohort study.

Abstract

The role of anticoagulation in systemic sclerosis (SSc) with precapillary pulmonary hypertension (PH) remains controversial, with conflicting evidence regarding its impact on survival, including potential harmful effects. Aiming to address this research gap, we evaluated both mortality and PH worsening in a large SSc-precapillary PH cohort, additionally accounting for SSc-specific risk factors in our analyses, to better inform individualized treatment decisions. This retrospective cohort study included SSc patients from the European Scleroderma Trials and Research database with precapillary PH confirmed by right heart catheterization. The association of OAC use with survival and PH worsening was assessed using Kaplan-Meier estimates and multivariable Cox regression models. To minimize confounding, propensity score matching was additionally performed. Among 614 patients included, 143 (23%) received OAC at the time of RHC. At baseline, patients on OAC had worse hemodynamic parameters. No significant association was found between OAC use and survival (HR 0.983 95%CI 0.724-1.334) or PH worsening (HR 1.070 95%CI 0.811-1.412). These findings remained consistent after propensity score matching and in the subgroup of 230 SSc patients with precapillary PH without interstitial lung disease. In this large SSc cohort with precapillary PH, we did not observe any clinical benefit associated with OAC, neither in terms of survival nor of PH worsening. Further prospective studies are needed to determine whether specific patient subgroups may benefit from anticoagulation therapy.