CF Centre, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany.
Dept of Pediatric Pulmonology, Allergy and Cystic Fibrosis, Children's Hospital, Christiane Herzog CF-Center, Goethe University, Frankfurt, Germany.
Eur Respir J. 2015 Aug;46(2):395-404. doi: 10.1183/09031936.00173514. Epub 2015 Apr 30.
Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy control subjects cross-sectionally and longitudinally and correlated these findings with lung function parameters.Cystic fibrosis patients with chronic fungal colonisation by Aspergillus fumigatus were characterised by an accumulation of a distinct granulocyte subset, expressing the HIV coreceptor CXCR4. Percentages of airway CXCR4(+) granulocytes correlated with lung disease severity in patients with cystic fibrosis.These studies demonstrate that chronic fungal colonisation with A. fumigatus in cystic fibrosis patients is associated with CXCR4(+) airway granulocytes, which may serve as a potential biomarker and therapeutic target in fungal cystic fibrosis lung disease.
囊性纤维化气道常被真菌定植。然而，这些真菌与免疫细胞的相互作用以及它们在囊性纤维化肺部疾病中的临床意义尚不完全清楚。我们对伴有和不伴有真菌定植、非囊性纤维化疾病对照和健康对照的囊性纤维化患者的气道液和外周血中的粒细胞进行了特征分析，进行了横断面和纵向研究，并将这些发现与肺功能参数相关联。患有慢性烟曲霉定植的囊性纤维化患者的特征是存在表达 HIV 核心受体 CXCR4 的特定粒细胞亚群的积累。气道 CXCR4(+)粒细胞的百分比与囊性纤维化患者的肺部疾病严重程度相关。这些研究表明，囊性纤维化患者的慢性烟曲霉定植与气道 CXCR4(+)粒细胞有关，这些粒细胞可能是真菌性囊性纤维化肺部疾病的潜在生物标志物和治疗靶点。
