New Insights in Microbial Species Predicting Lung Function Decline in CF: Lessons from the MucoFong Project.

Florian Lussac-Sorton,Michael Fayon,Florence Francis,Rodolphe Thiébaut,Marta Avalos-Fernandez,Perrine Soret,Stéphanie Bui,Raphael Enaud,Laurence Delhaes

doi:10.3390/jcm10163725

Abstract

Several predictive models have been proposed to understand the microbial risk factors associated with cystic fibrosis (CF) progression. Very few have integrated fungal airways colonisation, which is increasingly recognized as a key player regarding CF progression. To assess the association between the percent predicted forced expiratory volume in 1 s (ppFEV1) change and the fungi or bacteria identified in the sputum, 299 CF patients from the “MucoFong” project were included and followed-up with over two years. The relationship between the microorganisms identified in the sputum and ppFEV1 course of patients was longitudinally analysed. An adjusted linear mixed model analysis was performed to evaluate the effect of a transient or chronic bacterial and/or fungal colonisation at inclusion on the ppFEV1 change over a two-year period. Pseudomonas aeruginosa, Achromobacter xylosoxidans, Stenotrophomonas maltophilia, and Candida albicans were associated with a significant ppFEV1 decrease. No significant association was found with other fungal colonisations. In addition, the ppFEV1 outcome in our model was 11.26% lower in patients presenting with a transient colonisation with non-pneumoniae Streptococcus species compared to other patients. These results confirm recently published data and provide new insights into bacterial and fungal colonisation as key factors for the assessment of lung function decline in CF patients.

Highlights

Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting about 70,000 individuals in the world [1]
Our results were in agreement with previous findings indicating that several concomitant chronic bacterial colonisations at the time of diagnosis (P. aeruginosa, B. cepacia, and S. maltophilia) confer a worse ppFEV1 level in CF
Traditional CF pathogens remain the best predictors of disease outcomes in adults with CF [3,5,6,10,12,14,16,17,26,27,28,29,30,31,32,33]

Summary

Introduction

Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting about 70,000 individuals in the world [1] This multisystem disease affects primarily the lungs, causing repeated lung infections and chronic lung inflammation which lead to progressive respiratory failures associated with significant morbidity and early death. None but two of these studies [4,12] have taken into consideration fungal flora (mycobiota) of the CF airways These two studies were focused on managing spontaneous pneumothorax and scoring the risk of death or lung transplantation (LT) in CF, respectively [4,12], and provided data and scores that might be useful for daily practice but need to be used by other clinicians from other CF centres. Fungi are increasingly recognized as key players in the natural course of CF lung disease and as real threats in the case of lung transplantation (LT) [1,4,12,16,17,18]

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