Ivacaftor/lumacaftor/levonorgestrel/oral contraceptives interactions
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Ivacaftor/lumacaftor/levonorgestrel/oral contraceptives interactions Recrudescence of thoracic endometriosis syndrome, irregular menses and menorrhagia: case report
A woman in her early 20’s developed recrudescence of thoracic endometriosis syndrome in the form of catamenial haemoptysis, irregular menses and menorrhagia following concomitant administration ivacaftor/lumacaftor, levonorgestrel and unspecified combined oral contraceptive therapy [not all routes and outcomes stated; dosages not stated]. The nulligravid woman with cystic fibrosis had a history of catamenial haemoptysis (genotype F508del/F508del) since the age of 13 years. She had mild to moderate haemoptysis without bronchial artery embolisation or massive haemoptysis. Her bronchoscopy and imaging were postponed. At the age of 15 years, she started receiving unspecified combined oral contraceptive therapy for a presumed diagnosis of thoracic endometriosis syndrome. Over the next 7 years, her haemoptysis resolved. At 22 years of age, her baseline lung function decreased, predicted by recurrent pulmonary exacerbations. Hence, in 2015, she started receiving antibiotic treatment with ivacaftor/lumacaftor. Her lung function improved within the first year of therapy. However, she had a recrudescence of thoracic endometriosis syndrome in the form of catamenial haemoptysis associated menorrhagia and irregular menses. Therefore, the woman’s therapy with oral contraceptives was stopped, and the levonorgestrel-releasing intrauterine device (IUD) was inserted. Her treatment with ivacaftor/lumacaftor was continued because of constant improvement in lung function and pulmonary exacerbations. Inspite of IUD insertion, haemoptysis persisted with irregular menstrual bleeding. At her next menstrual cycle, a bronchoscopy and chest CT was performed. Chest CT showed ground-glass opacities at the right upper lobe and bilateral bronchiectasis. Bronchoscopy showed normal airways, except for tracheal hyperaemia and several erythematous lesions. A biopsy of the largest lesions was performed, and cytological analysis showed pulmonary alveolar macrophages and acute inflammation. Endometrial tissue was not found during the biopsy, but her presentation was suggestive of thoracic endometriosis syndrome. Hence, she was closely monitored in the reproductive endocrinology department. She also had completed 6 months course of leuprorelin [leuprolide acetate] for intermittent haemoptysis. During treatment with leuprorelin, haemoptysis resolved. In view of the reduced effectiveness of hormonal therapies due to ivacaftor/lumacaftor, her treatment with ivacaftor/lumacaftor was changed to ivacaftor/tezacaftor. After completion of leuprorelin therapy, IUD was removed. Subsequently, her combined oral contraceptive therapy was re-started. During treatment, she had no episodes of haemoptysis. In 2019, her therapy was switched to elexacaftor/ ivacaftor/tezacaftor with no recurrence of haemoptysis. Montemayor K, et al. Unmasking catamenial hemoptysis in the era of CFTR modulator therapy. Jo
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