结缔组织病相关肺动脉高压
Connective tissue disease-associated pulmonary arterial hypertension.
作者信息
Sung Yon K, Chung Lorinda
机构信息
Division of Pulmonary and Critical Care Medicine, Vera Moulton Wall Center for Pulmonary Vascular Disease, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA.
Division of Rheumatology and Immunology, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, CA 94305, USA; Division of Rheumatology, VA Palo Alto Health Care System, 3801 Miranda Avenue, Palo Alto, CA 94304, USA.
出版信息
Rheum Dis Clin North Am. 2015 May;41(2):295-313. doi: 10.1016/j.rdc.2015.01.003. Epub 2015 Feb 26.
Pulmonary arterial hypertension (PAH) is characterized by vascular remodeling of pulmonary arterioles that leads to increased pulmonary vascular resistance, right heart failure, and death. It is associated with connective tissue diseases, including systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. PAH is characterized by dyspnea on exertion and fatigue. Syncopal events suggest severe disease. Patients may present with signs of right heart failure. One- and 3-year survival rates are approximately 81% and 52%, respectively. Given the high prevalence and mortality, algorithms for screening are currently under investigation and will hopefully lead to earlier diagnosis and improved survival.
肺动脉高压（PAH）的特征是肺小动脉血管重塑，导致肺血管阻力增加、右心衰竭和死亡。它与结缔组织疾病相关，包括系统性硬化症、系统性红斑狼疮和混合性结缔组织病。PAH的特征是劳力性呼吸困难和疲劳。晕厥事件提示病情严重。患者可能出现右心衰竭的体征。1年和3年生存率分别约为81%和52%。鉴于其高患病率和死亡率，目前正在研究筛查算法，有望实现早期诊断并提高生存率。
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