A Clinical Trial of Anticoagulation in Scleroderma Heart-Lung Disease

硬皮病是一种自身免疫性疾病，可缩短30年以上的寿命。死亡的主要原因是肺动脉高压（PAH），这是一种肺血管压力升高的情况。即使在“先进”的多环芳烃治疗时代，也只有50%的患者存活超过5年。肺小血管血栓的形成在多环芳烃的发展中起着重要作用。在这项临床试验中，我们旨在确定抗凝治疗（防止凝血的治疗）是否能提高硬皮病PAH患者的生存率。

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.