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Unmasking catamenial hemoptysis in the era of CFTR modulator therapy
Journal of Cystic Fibrosis ( IF 5.4 ) Pub Date : 2020-07-01 , DOI: 10.1016/j.jcf.2020.01.005
Kristina Montemayor 1 , Abigail T Claudio 2 , Sara Carson 1 , Noah Lechtzin 1 , Mindy S Christianson 3 , Natalie E West 1
Affiliation  

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.

中文翻译:

CFTR 调节剂治疗时代揭示经期咯血

背景胸腔子宫内膜异位症(TES)是一种罕见的疾病,当子宫内膜异位症植入胸腔时,就会发生在女性身上。月经性咯血,即月经期间发生咯血,是 TES 公认的临床表现,通常采用激素治疗。病例摘要 我们提出了第一个记录在案的病例,描述了一名患有囊性纤维化 (CF) 的 25 岁女性在使用 Lumacaftor/Ivacaftor 后复发月经性咯血。讨论 我们回顾了有关 TES、药物管理和报道的囊性纤维化跨膜电导调节剂 (CFTR) 调节剂药物相互作用的文献。我们认为该患者经期咯血的复发是继发于 Lumacaftor/Ivacaftor 和口服避孕药治疗之间的药物相互作用。结论 我们的病例表明,患有经期咯血和 Tezacaftor/Ivacaftor 或 Elexacaftor/Tezacaftor/Ivacaftor 批准的基因突变的 CF 女性可以通过 CFTR 调节剂和激素避孕疗法得到有效治疗。
更新日期:2020-07-01
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