Should patients with systemic sclerosis‐related pulmonary arterial hypertension be anticoagulated?

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.