Fungi are increasingly recognised to have a significant role in the progression of lung disease in Cystic fibrosis with Aspergillus fumigatus the most common fungus isolated during respiratory sampling. The emergence of novel CFTR modulators has, however, significantly changed the outlook of disease progression in CF. In this review we discuss what impact novel CFTR modulators will have on fungal lung disease and its management in CF. We discuss how CFTR modulators affect antifungal innate immunity and consider the impact of Ivacaftor on fungal disease in individuals with gating mutations. We further review the increasing complication of drug–drug interactions with concurrent use of azole antifungal medication and highlight key unknowns that require addressing to fully understand the impact of CFTR modulators on fungal disease.

越来越多的人认识到真菌在烟曲霉囊性纤维化肺部疾病的进展中具有重要作用呼吸道采样中分离出的最常见的真菌。然而，新型 CFTR 调节剂的出现显着改变了 CF 疾病进展的前景。在这篇综述中，我们讨论了新型 CFTR 调节剂对真菌性肺病及其在 CF 中的管理的影响。我们讨论了 CFTR 调节剂如何影响抗真菌先天免疫，并考虑 Ivacaftor 对具有门控突变的个体的真菌疾病的影响。我们进一步回顾了与唑类抗真菌药物同时使用的药物相互作用日益增加的并发症，并强调了需要解决的关键未知因素，以充分了解 CFTR 调节剂对真菌疾病的影响。