A Clinical Trial of Anticoagulation in Scleroderma Heart-Lung Disease

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Scleroderma is an autoimmune disease that shortens life span by over 30 years. The main cause of death is pulmonary arterial hypertension (PAH), a condition of increased pressure in the lung vessels. Even in the era of 'advanced' PAH therapy, only 50% of patients are alive beyond 5 years. Formation of clots in the small blood vessels of the lung plays a major role in the development of PAH. In this clinical trial we aim to determine whether anticoagulation (treatment to prevent clotting) improves survival in scleroderma PAH.