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特发性肺动脉高压(IPAH)是指原因不明的肺血管阻力增加引起持续性肺动脉压力升高,其病理改变为肺血管的收缩和重塑、原位血栓形成和血管内皮损伤。由于缺乏有效的治疗措施,病死率极高,预后差,明确诊断后平均存活时间为2~3年。近年来随着对IPAH的分子生物学水平研究的深入,一些新的治疗方法如前列环素及其衍生物、内皮素受体拮抗剂等不断应用于临床,使IPAH的生存率明显提高,生活质量明显改善。而一些被证明对IPAH具有潜在治疗意义的药物如肾上腺髓质素、西地那非、他汀类药物等仍在临床研究中,在不久的将来可能成为治疗IPAH的一线用药。
Idiopathic Pulmonary Hypertension (IPAH) refers to an unexplained increase in pulmonary vascular resistance that causes persistent pulmonary artery pressure elevation. Its pathological changes are pulmonary vasoconstriction and remodeling, in situ thrombosis, and vascular endothelial injury. Due to the lack of effective treatment measures, high mortality, poor prognosis, a clear diagnosis of the average survival time of 2 to 3 years. In recent years, with the further research on the molecular biology of IPAH, some new therapies such as prostacyclin and its derivatives, endothelin receptor antagonists continue to be used clinically, so that the survival rate of IPAH significantly increased, life Quality improved significantly. Some drugs that have been shown to have potential therapeutic implications for IPAH, such as adrenomedullin, sildenafil, statins and the like, are still in clinical study and may become the first-line treatment for IPAH in the near future.