虽然肺动脉高压仍属罕见疾病,但随着认识的不断增加,估计的患病率为每百万成年居民 15.0 例,年发病率为每百万成年居民 2.4 例。[6]Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023-1030.http://www.atsjournals.org/doi/full/10.1164/rccm.200510-1668OC#.UnPnTfnxobAhttp://www.ncbi.nlm.nih.gov/pubmed/16456139?tool=bestpractice.com疾病的年龄和性别分布显示随着时间的推移而发生变化。在美国国立卫生研究院(NIH) 20 世纪 80 年代的登记中,录入患者的平均年龄是 36 岁,女性与男性比是 1.7。[7]Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107:216-223.http://www.ncbi.nlm.nih.gov/pubmed/3605900?tool=bestpractice.com当代的法国和美国登记结果显示,确诊时的平均年龄是 50 岁。[6]Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023-1030.http://www.atsjournals.org/doi/full/10.1164/rccm.200510-1668OC#.UnPnTfnxobAhttp://www.ncbi.nlm.nih.gov/pubmed/16456139?tool=bestpractice.com[8]Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376-387.http://www.ncbi.nlm.nih.gov/pubmed/19837821?tool=bestpractice.com 有一定比例的患者(法国登记数据:9%)年龄超过 70 岁。虽然法国登记数据证实女性与男性比为 1.6,[6]Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023-1030.http://www.atsjournals.org/doi/full/10.1164/rccm.200510-1668OC#.UnPnTfnxobAhttp://www.ncbi.nlm.nih.gov/pubmed/16456139?tool=bestpractice.com 但美国登记数据显示了更高的女性患病率,女性与男性比达到 3.9。[8]Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376-387.http://www.ncbi.nlm.nih.gov/pubmed/19837821?tool=bestpractice.com重要的是,根据目前的美国登记数据,20 世纪 80 年代 NIH 登记所显示的 IPAH 明显诊断延迟的状况仍未改变(症状发生至确诊的中位间期是 1.1 年)。[8]Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376-387.http://www.ncbi.nlm.nih.gov/pubmed/19837821?tool=bestpractice.com