Leveraging early markers of cystic fibrosis structural lung disease to improve outcomes

[摘要] The natural history of cystic fibrosis (CF) lung disease has been described as a complex process of obstruction, infection, and inflammation, all contributing to airways injury and eventual structural disease (i.e. bronchiectasis). Consequences of the CF genetic abnormality begin in utero and are the basis for newborn screening for elevated levels of circulating immunoreactive trypsinogen (IRT) [1]. Thus, it should be no surprise that the process of airway obstruction begins immediately at birth. Ample cross-sectional evidence has been published of airway obstruction, inflammation, bacterial infection, bronchial wall thickening, air trapping and bronchiectasis in the first year of life for infants with CF [2]. Despite a wealth of observational data, there remain uncertainties as to possible/probable relationships between these different early complications. For instance, there remains some debate as to whether inflammation is inherent to the CF lung, a secondary result of opportunistic microbial infection, or both. Similarly, although there has been an association noted between infection and bronchiectasis in children, reported discordance between the sites of infection and the anatomical site of early bronchiectasis suggest they may not be directly linked [3]. An original study published in this issue of the European Respiratory Journal has now demonstrated that early radiographic lung abnormalities are indeed associated with later structural disease in children with CF [4]. A notable contribution of this work is that of quantitating specific biomarkers of future lung disease risk: atelectasis, airway wall thickening, and increased interleukin (IL)-8 concentrations.

[学科分类] 呼吸医学