Abstract
Cystic fibrosis (CF), the most common autosomal-recessive genetic disease in the Caucasian population, is characterized by frequent respiratory infections and progressive lung disease. Fungal species are commonly found in patients with CF, and among them, Aspergillus fumigatus is the most frequently isolated. While bacteria, particularly Pseudomonas aeruginosa, have a well-established negative effect on CF lung disease, the impact of fungal infections remains unclear. In patients with CF, inhalation of Aspergillus conidia can cause allergic bronchopulmonary aspergillosis (ABPA), a Th2-mediated lung disease that can contribute to disease progression. Clinical features, diagnostic criteria and treatment of ABPA are still a matter of debate. Given the consequences of a late ABPA diagnosis or the risk of ABPA overdiagnosis, it is imperative that the diagnostic criteria guidelines are reviewed and standardized. Along with traditional criteria, radiological features are emerging as tools for further classification as well as novel immunological tests. Corticosteroids, itraconazole and voriconazole continue to be the bedrock of ABPA therapy, but other molecules, such as posaconazole, vitamin D, recombinant INF-γ and Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators, have been showing positive results. However, few studies have been conducted recruiting CF patients, and more research is needed to improve the prevention and the classification of clinical manifestations as well as to personalize treatment. Early recognition and early treatment of fungal infections may be fundamental to prevent progression of CF disease. The aim of this narrative review is to give an update on ABPA in children with CF.
Highlights
Aspergillus species are ubiquitous saprophytic fungi found in water, soils, organic decay and in indoor environments
Those with P. aeruginosa, have a well-established negative effect on cystic fibrosis (CF) lung disease, the impact of fungal infections remains unclear [2], but an early diagnosis of allergic bronchopulmonary aspergillosis (ABPA) seems clinically relevant in order to avoid deterioration of lung function [3]
Reece et al [6] demonstrated that an extended incubation period and the use of quantitative polymerase chain reaction increased the detection of A. fumigatus in sputum samples from 16 to 52% of patients compared to the routine culture method
Summary
Aspergillus species are ubiquitous saprophytic fungi found in water, soils, organic decay and in indoor environments. The commonest species associated to respiratory disease in humans is Aspergillus fumigatus. Aspergillus spores are inhaled daily, usually with no consequences, but, in susceptible people with pulmonary diseases such as cystic fibrosis (CF), the inhalation of Aspergillus conidia can colonize the lung and cause allergic bronchopulmonary aspergillosis (ABPA) [1]. ABPA is a Th2-mediated lung disease caused by hypersensitivity to Aspergillus hyphae that carries significant morbidity for patients with CF and may progress to bronchiectasis and pulmonary fibrosis. The aim of this narrative review is to give an update on ABPA in children with CF, regarding the newest diagnostic tools and therapeutic approaches
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