Cystic Fibrosis Mice Develop Spontaneous Chronic Bordetella Airway Infections.

Chronic pulmonary disease and infection is the primary cause of morbidity and mortality in people with cystic fibrosis (CF). Though Pseudomonas aeruginosa, is most commonly found in the airways of individuals with CF, there is increasing appreciation for the diversity of the CF microbiome, including other taxa such as Bordetella. Here we describe the identification and impact of Bordetella pseudohinzii infection in CF mice, which previously have not been thought to develop spontaneous airway infections. We determined that CF mice are more susceptible to the B. pseudohinzii infections, and less able to resolve the infection than non-CF mice. Moreover, in both CF and non-CF mice, B. pseudohinzii infections lead to markedly reduced respiratory rates and a CF-specific immune response. These results establish the CF mouse model as an important tool for the study of CF-relevant infection and highlight the potential contribution of Bordetella to CF clinical pathology.