Does anticoagulant therapy improve survival of patients with systemic sclerosis-associated PAH?

Answer:

Early studies suggested a possible mortality benefit for anticoagulation with warfarin in patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH). However, a post-hoc analysis of a PAH registry study reported a trend towards worse survival in patients with SSc-PAH who received anticoagulant therapy compared with those not receiving anticoagulant therapy [1]. A subsequent analysis of a separate registry of PAH patients also reported an increased mortality in the same population, even after adjustment for confounders, including disease severity [2]. Based on findings of these studies, anticoagulant therapy should not be administered to patients with systemic sclerosis-associated PAH.

Reference

1. Olsson KM, Delcroix M, Ghofrani HA, et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014; 129:57.
2. Preston IR, Roberts KE, Miller DP, et al. Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL). Circulation 2015; 132:2403.