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Updated: May 11, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Should patients with systemic sclerosis-related pulmonary arterial hypertension be anticoagulated?
M Nikpour1, W Stevens, S M Proudman
1Department of Medicine, St Vincent's Academic Centre, The University of Melbourne, Melbourne, Victoria, Australia. mnikpour@medstv.unimelb.edu.au
Abstract:
Pulmonary arterial hypertension (PAH) is a major cause of mortality in scleroderma and despite 'advanced' therapies confers a median survival of less than 5 years. Anticoagulation in systemic sclerosis-related PAH (SSc-PAH) is currently one of the most contentious issues in the management of patients with connective tissue disease. While some studies have shown a survival benefit with warfarin therapy in this disease, others have not. Accordingly, a state of clinical equipoise exists in relation to anticoagulation in SSc-PAH. With an over fivefold reduction in mortality demonstrated in some observational studies, the issue of anticoagulation in SSc-PAH demands resolution through a well-designed randomised controlled trial.
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