Background

There are important advances in the management of bacterial infection in patients with cystic fibrosis (CF), but there are many gaps in the field of fungal infections.

Aims

The aim of this study was to analyse whether chronic respiratory filamentous fungal colonization had clinical impact and whether antifungal treatment can change the disease.

Methods

The prospective, bicentric and descriptive study was carried out within a 3-year follow-up period, with four-month periodicity medical controls. Adult patients from two CF units of tertiary hospitals were included. Clinical, microbiological, analytical and spirometric variables were collected. Quality of life was evaluated in a subgroup, using the Spanish version of the Revised Cystic Fibrosis Quality of Life Questionnaire (CFQ-R). To statistically analyze the evolution of forced expiratory along time (volume of air blown out in 1 second -FEV₁-) and the forced vital capacity (FVC), mixed linear models were carried out.

Results

From the ninety-eight patients under study, 40 suffered chronic filamentous fungal colonization. The presence of filamentous fungi in airway was associated to an annual fall of FEV₁ and FVC of 0.029 and 0.017 litres, respectively (p<0.001). In addition, worse quality of life based on CFQ-R, significant when concerning physical condition and emotional state, was also linked with the fungal colonization. Protocolized antifungal therapy, nebulized or oral, improved FEV₁ in 0.023 and 0.024 litres per year, respectively (p<0.001).

Conclusions

Chronic filamentous fungal colonization in patients with CF is associated with a significant annual decline of lung function that persists over time. Chronic antifungal therapy slows down this progression, mainly in the patient with more advanced disease.

中文翻译：

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囊性纤维化患者气道中的丝状真菌：只是旁观者？

背景

囊性纤维化（CF）患者细菌感染的管理取得了重要进展，但真菌感染领域仍有许多空白。

宗旨

本研究的目的是分析慢性呼吸道丝状真菌定植是否具有临床影响以及抗真菌治疗是否可以改变这种疾病。

方法

前瞻性、双中心和描述性研究是在 3 年随访期内进行的，医学控制周期为 4 个月。包括来自三级医院的两个CF单位的成年患者。收集了临床、微生物学、分析和肺活量变量。使用西班牙语版本的修订版囊性纤维化生活质量问卷 (CFQ-R) 在一个亚组中评估生活质量。为了统计分析用力呼气随时间的演变（1 秒内吹出的空气量 -FEV ₁ -）和用力肺活量 (FVC)，进行了混合线性模型。

结果

在接受研究的 98 名患者中，40 名患有慢性丝状真菌定植。气道中丝状真菌的存在与 FEV ₁和 FVC 分别下降0.029 和 0.017 升（p<0.001）有关。此外，基于 CFQ-R 的较差生活质量（在涉及身体状况和情绪状态时很重要）也与真菌定植有关。规定的抗真菌治疗，雾化或口服，FEV ₁每年分别提高0.023 和 0.024 升（p<0.001）。

结论

CF 患者的慢性丝状真菌定植与肺功能的显着年度下降有关，并且随着时间的推移持续存在。慢性抗真菌治疗减缓了这种进展，主要是在患有更晚期疾病的患者中。