结缔组织病相关肺动脉高压
Pulmonary arterial hypertension in connective tissue diseases.
机构信息
Pulmonary Hypertension Program, Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, 1830 East Monument Street, Baltimore, MD 21205, USA.
出版信息
Heart Fail Clin. 2012 Jul;8(3):413-25. doi: 10.1016/j.hfc.2012.04.001.
Abstract
Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs). Approximately 10% of patients with systemic sclerosis develop PAH, the prevalence being much lower in other CTDs. However, PAH is an important contributor to morbidity and mortality in all forms of CTD. Despite similarities in presentation, hemodynamic perturbations, and pathogenesis, patients with CTD-associated PAH (CTD-PAH) usually have a poorer response to PAH-specific medications and poorer prognosis than patients with idiopathic PAH (IPAH). Select patients with CTD-PAH may be candidates for lung transplantation, but results are less favorable than for IPAH because of comorbidities and complications specifically associated with CTD.
摘要
肺动脉高压（PAH）可能会使多种结缔组织疾病（CTD）复杂化。大约 10%的系统性硬化症患者会发生 PAH，而在其他 CTD 中，患病率要低得多。然而，PAH 是所有形式的 CTD 发病率和死亡率的重要因素。尽管在表现、血流动力学改变和发病机制上存在相似之处，但与特发性 PAH（IPAH）患者相比，CTD 相关 PAH（CTD-PAH）患者对 PAH 特异性药物的反应通常较差，预后也较差。少数 CTD-PAH 患者可能是肺移植的候选者，但由于与 CTD 相关的合并症和并发症，结果不如 IPAH 好。
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