In the past three decades, fungal respiratory colonization and fungal respiratory infections increasingly raised concern in cystic fibrosis (CF). Reasons for this are a better knowledge of the pathogenicity of fungi, whereby detection is sought in more and more CF centers, but also improvement of detection methods. However, differences in fungal detection rates within and between geographical regions exist and indicate the need for standardization of mycological examination of respiratory secretions. The still existing lack of standardization also complicates the assessment of fungal pathogenicity, relevance of fungal detection and risk factors for fungal infections. Nevertheless, numerous studies have now been conducted on differences in detection methods, epidemiology, risk factors, pathogenicity and therapy of fungal diseases in CF. Meanwhile, some research groups now have classified fungal disease entities in CF and developed diagnostic criteria as well as therapeutic guidelines.The following review presents an overview on fungal species relevant in CF. Cultural detection methods with their respective success rates as well as susceptibility testing will be presented, and the problem of increasing azole resistance in Aspergillus fumigatus will be highlighted. Next, current data and conflicting evidence on the epidemiology and risk factors for fungal diseases in patients with CF will be discussed. Finally, an overview of fungal disease entities in CF with their current definitions, diagnostic criteria and therapeutic options will be presented.

在过去的三十年中，真菌呼吸道定植和真菌呼吸道感染越来越引起囊性纤维化 (CF) 的关注。这样做的原因是对真菌致病性的更好了解，从而在越来越多的 CF 中心寻求检测，同时也改进了检测方法。然而，地理区域内和地理区域之间的真菌检测率存在差异，这表明需要对呼吸道分泌物进行真菌学检查标准化。仍然缺乏标准化也使真菌致病性、真菌检测的相关性和真菌感染的危险因素的评估变得复杂。尽管如此，现在已经对 CF 真菌病的检测方法、流行病学、危险因素、致病性和治疗方法的差异进行了大量研究。同时，一些研究小组现在已经对 CF 中的真菌疾病实体进行了分类，并制定了诊断标准和治疗指南。以下综述概述了与 CF 相关的真菌物种。将介绍具有各自成功率的培养检测方法以及药敏试验，以及增加唑类耐药性的问题烟曲霉将突出显示。接下来，将讨论关于 CF 患者真菌病的流行病学和危险因素的当前数据和相互矛盾的证据。最后，将介绍 CF 中真菌疾病实体的概述及其当前定义、诊断标准和治疗选择。