Abstract
Background: Aggressive treatment of primary ciliary dyskinesia (PCD) often stabilizes spirometry over time. We hypothesized that spirometry may overestimate the degree of stability.
Methods: Twenty PCD patients (median age, 11.6 yrs; range, 6.5-27.5 yrs) underwent spirometry and HRCT at median 2.3 yrs interval. The first evaluation was made in stable state and the second because of deterioration unresponsive to standard therapy. CT scans were scored blind by two experienced raters.
Results: At the second evaluation, spirometry did not change while CT scores significantly worsened (p<0.01).
Age was significantly related to CT total score (r=0.5; p=0.02). At both evaluations CT total score was related with FEV1 Z score (r= –0.5; p=0.015 and r= –0.7; p= 0.001, respectively) and FVC Z score (r= –0.6; p= 0.006 and r= -0.7; p=0.001, respectively). No relationship was found between the change in CT scores and the change in spirometry.
Conclusions: In PCD patients with pulmonary deterioration, spirometry may fail to detect worsening CT findings. Structural changes may progress with stable spirometry, calling into question the usefulness of serial spirometries to monitor PCD lung disease.
- © 2011 ERS