A Multi-Centre Double-Blind Randomised Placebo-Controlled Trial of Oral Anticoagulation in Systemic Sclerosis-Related Pulmonary Arterial Hypertension

硬皮病是一种自身免疫性疾病，会缩短30多年的寿命。死亡的主要原因是肺动脉高压(PAH)，这是一种肺血管压力增加的情况。即使在“高级”PAH治疗的时代，也只有50%的患者能活到5年以上。肺小血管内凝块的形成在PAH的发生发展中起主要作用。在这项临床试验中，我们的目标是确定抗凝(防止凝血的治疗)是否可以提高硬皮病PAH的存活率。

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.