Almaaitah Saja, Highland Kristin B, Tonelli Adriano R
Medicine Institute, Cleveland Clinic, Cleveland, OH, USA.
Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, OH, USA.
Integr Blood Press Control. 2020 Mar 23;13:15-29. doi: 10.2147/IBPC.S232038. eCollection 2020.
Systemic sclerosis (SSc) is a rare and complex immune-mediated connective tissue disease characterized by multi-organ fibrosis and dysfunction. Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a leading cause of death in this population. Pulmonary arterial hypertension (PAH) can coexist with other forms of pulmonary hypertension in SSc, including pulmonary hypertension related to left heart disease, interstitial lung disease, chronic thromboembolism and pulmonary venous occlusive disease, which further complicates diagnosis and management. Available pulmonary arterial hypertension therapies target the nitric oxide, endothelin and prostacyclin pathways. These therapies have been studied in SSc-PAH in addition to idiopathic PAH, often with different treatment responses. In this article, we discuss the management as well as the treatment options for patients with SSc-PAH.
系统性硬化症（SSc）是一种罕见且复杂的免疫介导的结缔组织疾病，其特征为多器官纤维化和功能障碍。系统性硬化症相关肺动脉高压（SSc-PAH）是该人群的主要死亡原因。肺动脉高压（PAH）可与SSc中的其他形式的肺动脉高压共存，包括与左心疾病、间质性肺疾病、慢性血栓栓塞和肺静脉闭塞性疾病相关的肺动脉高压，这进一步使诊断和管理复杂化。现有的肺动脉高压治疗方法针对一氧化氮、内皮素和前列环素途径。除特发性PAH外，这些疗法也在SSc-PAH中进行了研究，通常有不同的治疗反应。在本文中，我们讨论了SSc-PAH患者的管理以及治疗选择。
