Department of Pathophysiology and Transplantation, University of Milan, Italy; Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Department of Pathophysiology and Transplantation, University of Milan, Italy; Internal Medicine Department, Respiratory Unit and Cystic Fibrosis Adult Center. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
J Cyst Fibros. 2022 Sep;21(5):885-887. doi: 10.1016/j.jcf.2022.04.010. Epub 2022 Apr 18.
Elexacaftor/tezacaftor/ivacaftor (ETI) is associated with major improvements in respiratory outcomes of individuals with cystic fibrosis (CF) and at least one Phe508del mutation. Although ETI was well tolerated in registration studies, the attention on adverse events not previously described is very high in the post-marketing phase. In this case series we report the onset of systemic arterial hypertension in 4 individuals with CF within the first weeks of starting therapy. All patients needed cardiac evaluation and started chronic anti-hypertensive therapy. Until more data is available, this report could foster the attention of CF physicians towards careful monitoring of cardiovascular parameters in patients starting ETI.
依利卓（Elexacaftor）/泰他西普（Tezacaftor）/依伐卡托（Ivacaftor）（ETI）可显著改善至少携带一个 F508del 突变的囊性纤维化（CF）患者的呼吸系统结局。尽管 ETI 在注册研究中具有良好的耐受性，但在上市后阶段，人们对以前未描述过的不良事件的关注度非常高。在本病例系列中，我们报告了 4 名 CF 患者在开始治疗的最初几周内出现全身动脉高血压。所有患者均需要进行心脏评估，并开始接受慢性抗高血压治疗。在获得更多数据之前，本报告可能会促使 CF 医生更加关注开始接受 ETI 治疗的患者的心血管参数的监测。
