揭开 CFTR 调节剂治疗时代的月经性咯血的面纱。
Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.
机构信息
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University, 1830 E. Monument St 5th Floor, Baltimore, MD 21205, United States.
Cystic Fibrosis Foundation, Bethesda, MD, United States.
出版信息
J Cyst Fibros. 2020 Jul;19(4):e25-e27. doi: 10.1016/j.jcf.2020.01.005. Epub 2020 Jan 24.
BACKGROUND
Thoracic endometriosis syndrome (TES) is a rare condition that occurs in women when endometriosis implants into the thoracic cavity. Catamenial hemoptysis, the occurrence of hemoptysis with menstruation, is a recognized clinical manifestation of TES commonly treated with hormonal therapy.
CASE SUMMARY
We present the first documented case describing the recrudescence of catamenial hemoptysis in the setting of Lumacaftor/Ivacaftor administration in a 25-year-old woman with cystic fibrosis (CF).
DISCUSSION
We review the literature on TES, pharmacologic management, and reported cystic fibrosis transmembrane conductance regulator (CFTR) modulator drug interactions. We propose that our patient's recrudescence of catamenial hemoptysis was secondary to a drug-drug interaction between Lumacaftor/Ivacaftor and oral contraceptive therapy.
CONCLUSION
Our case suggests that women with CF who have catamenial hemoptysis and a genetic mutation approved for Tezacaftor/Ivacaftor or Elexacaftor/Tezacaftor/Ivacaftor can be managed effectively with either CFTR modulator and hormonal contraceptive therapy.
背景
胸子宫内膜异位症综合征（TES）是一种罕见的病症，发生在子宫内膜异位症植入胸腔的女性中。月经性咯血，即随月经出现咯血，是 TES 的一种公认的临床表现，通常采用激素治疗进行治疗。
病例总结
我们报告了首例在囊性纤维化（CF）患者中使用 Lumacaftor/Ivacaftor 治疗时出现月经性咯血复发的病例。
讨论
我们回顾了 TES、药物治疗以及已报道的囊性纤维化跨膜电导调节因子（CFTR）调节剂药物相互作用的文献。我们提出，我们患者的月经性咯血复发是 Lumacaftor/Ivacaftor 和口服避孕药治疗之间药物相互作用的结果。
结论
我们的病例表明，有月经性咯血且有基因变异的 CF 女性可以通过 CFTR 调节剂和激素避孕治疗进行有效管理。
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