Division of Pulmonary Circulation, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Osaka, Japan.
Allergol Int. 2011 Dec;60(4):419-24. doi: 10.2332/allergolint.11-RAI-0362. Epub 2011 Oct 25.
Pulmonary hypertension was defined as mean pulmonary artery pressure ≥ 25 mmHg at the 4th World Symposium on Pulmonary Hypertension. In 2009, the European Society of Cardiology and European Respiratory Society jointly created guidelines for practical pulmonary hypertension classifications and treatments based on the discussions at the 4th World Symposium. This classification is characterized by division into five groups: Pulmonary arterial hypertension (PAH); Pulmonary hypertension due to left heart disease; Pulmonary hypertension due to lung disease and/or hypoxia; Chronic thromboembolic pulmonary hypertension; and Pulmonary hypertension with unclear and/or multifactorial mechanisms. PAH is a common and fatal complication of connective tissue disease (CTD), but pulmonary hypertension in CTD consists of PAH, pulmonary hypertension caused by myocardial involvement, pulmonary veno-occlusive disorder, pulmonary hypertension due to interstitial lung disease. PAH has been studied widely in SSc and the estimated prevalence of 7-12%. Treatment of CTD associated PAH (CTD-PAH) consists of general therapeutic options and specific treatment. Specific treatment of CTD-PAH patients is targeted to produce vasodilatation. Calcium channel blockers (CCBs) are indicated in cases where a sufficient decrease in pulmonary arterial pressure is seen in vasoreactivity testing. If vasoreactivity is absent in CTD-PAH patients, the treatment consists of the endothelin receptor antagonists, the prostacyclin analogues and phosphodiesterase-type 5 inhibitors. Few data are available to support the use of immunosuppression in CTD-PAH. However, some case reports suggested that a minority of CTD-PAH patients could benefit from immunosuppressive therapy. The treatment of CTD-PAH patients may differ from the treatment of idiopathic PAH.
肺动脉高压定义为第 4 届世界肺动脉高压研讨会上平均肺动脉压≥25mmHg。2009 年，欧洲心脏病学会和欧洲呼吸学会共同根据第 4 届世界肺动脉高压研讨会的讨论结果制定了实用肺动脉高压分类和治疗指南。这种分类的特点是分为五组：肺动脉高压（PAH）；左心疾病引起的肺动脉高压；肺部疾病和/或缺氧引起的肺动脉高压；慢性血栓栓塞性肺动脉高压；以及原因不明和/或多因素机制引起的肺动脉高压。PAH 是结缔组织病（CTD）的常见且致命的并发症，但 CTD 中的肺动脉高压包括 PAH、心肌受累引起的肺动脉高压、肺静脉闭塞性疾病、间质性肺病引起的肺动脉高压。SSc 中广泛研究了 PAH，估计患病率为 7-12%。CTD 相关 PAH（CTD-PAH）的治疗包括一般治疗选择和特定治疗。CTD-PAH 患者的特定治疗旨在产生血管扩张。钙通道阻滞剂（CCB）适用于血管反应性试验中观察到肺动脉压充分降低的情况。如果 CTD-PAH 患者的血管反应性缺失，则治疗包括内皮素受体拮抗剂、前列腺素类似物和磷酸二酯酶-5 抑制剂。有少量数据支持在 CTD-PAH 中使用免疫抑制。然而，一些病例报告表明，少数 CTD-PAH 患者可能受益于免疫抑制治疗。CTD-PAH 患者的治疗可能与特发性 PAH 的治疗不同。
