Brown Zoe R, Nikpour Mandana
Department of Medicine, The University of Melbourne, Victoria, Australia.
Clinic of Rheumatology, St. Vincent's Hospital, Melbourne, Victoria, Australia.
Eur J Rheumatol. 2020 Oct;7(Suppl 3):S187-S192. doi: 10.5152/eurjrheum.2020.19114. Epub 2020 Oct 1.
Systemic sclerosis (SSc), a chronic multisystem autoimmune disease characterized by fibrosis of the skin and internal organs and vasculopathy, has a high burden of mortality. One of the major contributors to mortality in patients with SSc is pulmonary arterial hypertension (PAH), which affects up to 10% of individuals and results in up to 15 years of life loss. Best practice recommendations are for asymptomatic patients with SSc and SSc-spectrum disorder to be screened annually for the early detection of SSc-PAH. Recently published data from large registries have shown improvements in the long-term outcomes in patients who are diagnosed with SSc-PAH because of systematic annual screening. This review will address the current clinical and research implications of the screening for the early detection of SSc-PAH.
系统性硬化症（SSc）是一种慢性多系统自身免疫性疾病，其特征为皮肤和内脏器官纤维化以及血管病变，死亡率很高。SSc患者死亡的主要原因之一是肺动脉高压（PAH），PAH影响高达10%的患者，并导致多达15年的生命损失。最佳实践建议是，对无症状的SSc患者和SSc谱系障碍患者每年进行筛查，以便早期发现SSc相关的PAH。大型登记处最近公布的数据显示，由于进行了系统的年度筛查，被诊断为SSc相关PAH的患者的长期预后有所改善。本综述将探讨早期检测SSc相关PAH的筛查在当前临床和研究中的意义。
