A cross-sectional analysis of 408 paediatric and adult patients with primary ciliary dyskinesia from 12 countries clearly showed that the detection of Pseudomonas aeruginosa in the respiratory tract is associated with significantly poorer lung function than in patients without this pathogen.
Interestingly, the age of the affected individuals appears to play a role, as no clear correlation between the detection of Pseudomonas aeruginosa and impaired lung function was found in the subgroup of children.
In contrast to Pseudomonas aeruginosa, other frequently isolated pathogens such as Haemophilus influenzae, MSSA and Streptococcus pneumoniae were not associated with reduced lung function in the entire collective. This could indicate that not all bacterial infections in primary ciliary dyskinesia are of equal clinical relevance.
The cross-sectional analysis of the PROVALF-PCD cohort suggests that regular microbiological checks should be carried out in individuals with primary ciliary dyskinesia in order to detect Pseudomonas aeruginosa at an early stage. The study authors emphasise that, if this pathogen is detected, early eradication therapy and immediate treatment of exacerbations in colonised patients are advisable.
This recommendation is in line with the recently published international consensus paper on infection prevention and control in primary ciliary dyskinesia. The experts recommend the eradication of Pseudomonas aeruginosa if the pathogen is detected – even in asymptomatic cases. Haemophilus influenzae and MSSA, on the other hand, should only be treated if symptoms are present.
The study shows an association between Pseudomonas aeruginosa and poorer lung function, but a causal relationship cannot be proven due to the cross-sectional design. Therefore, prospective, longitudinal studies are necessary to clarify the causal relationship between bacterial infections and the course of lung function in primary ciliary dyskinesia.