Background: The balance between matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs)
is important in the regulation of airway damage.
Objective: To evaluate whether they are important in the pathophysiology of primary and secondary
ciliary dyskinesia (PCD, SCD).
Methods: We measured sputum bacteriology, lung CT changes, MMPs, TIMPs and lung function in 86
patients (51 PCD, 35 SCD) in a cross-sectional study; the 10 controls studied did not have HRCT or sputum
cultures. MMPs, TIMPs and lung function were evaluated longitudinally for up to one year in 38 PCD
patients.
Results: At baseline, there were no differences in MMPs, TIMPs and MMPs/TIMPs, between PCD and SCD
but lower levels were found in controls. There was an association between poorer lung function with
increasing levels of MMPs in PCD, while in SCD only MMP-9/TIMP-1 values correlated with FRC z-scores.
Levels of MMPs and TIMPs significantly correlated with severity HRCT changes. Longitudinally, there
were significant correlations between slope of changes in spirometric parameters and slope of change in
sputum MMPs in PCD patients.
Conclusions: In conclusion, we report for the first time that increased MMPs are associated with worse
airway damage in PCD and SCD, and thus are potential therapeutic targets