Tio Darryl, Leter Edward, Boerrigter Bart, Boonstra Anco, Vonk-Noordegraaf Anton, Bogaard Harm Jan
Pulmonology Department, VU University Medical Center, Amsterdam, The Netherlands.
PLoS One. 2013 Oct 23;8(10):e78132. doi: 10.1371/journal.pone.0078132. eCollection 2013.
When hemoptysis complicates pulmonary arterial hypertension (PAH), it is assumed to result from bronchial artery hypertrophy. In heritable PAH, the most common mutation is in the BMPR2 gene, which regulates growth, differentiation and apoptosis of mesenchymal cells. The aim of this study is to determine the relationship in PAH between the occurrence of hemoptysis, and disease progression, bronchial artery hypertrophy, pulmonary artery dilation and BMPR2 mutations.
129 IPAH patients underwent baseline pulmonary imaging (CT angio or MRI) and repeated right-sided heart catheterization. Gene mutations were assessed in a subset of patients.
Hemoptysis was associated with a greater presence of hypertrophic bronchial arteries and more rapid hemodynamic deterioration. The presence of a BMPR2 mutation did not predispose to the development of hemoptysis, but was associated with a greater number of hypertrophic bronchial arteries and a worse baseline hemodynamic profile.
Hemoptysis in PAH is associated with bronchial artery hypertrophy and faster disease progression. Although the presence of a BMPR2 mutation did not correlate with a greater incidence of hemoptysis in our patient cohort, its association with worse hemodynamics and a trend of greater bronchial arterial hypertrophy may increase the risk of hemoptysis.
当咯血使肺动脉高压（PAH）病情复杂化时，一般认为是由支气管动脉肥大所致。在遗传性PAH中，最常见的突变存在于BMPR2基因，该基因调控间充质细胞的生长、分化和凋亡。本研究的目的是确定PAH患者咯血的发生与疾病进展、支气管动脉肥大、肺动脉扩张及BMPR2突变之间的关系。
129例特发性PAH患者接受了基线肺部成像检查（CT血管造影或MRI）及重复的右心导管检查。对部分患者进行了基因突变评估。
咯血与肥大的支气管动脉较多以及血流动力学更快恶化相关。BMPR2突变的存在并非咯血发生的诱因，但与较多的肥大支气管动脉及更差的基线血流动力学特征相关。
PAH患者的咯血与支气管动脉肥大及更快的疾病进展相关。尽管在我们的患者队列中，BMPR2突变的存在与咯血发生率较高并无关联，但其与更差的血流动力学及支气管动脉肥大加剧的趋势可能会增加咯血风险。
