Lung disease assessment in primary ciliary dyskinesia: a comparison between chest high-field magnetic resonance imaging and high-resolution computed tomography findings.
BACKGROUND
Primary ciliary dyskinesia (PCD) is associated with pulmonary involvement that requires periodical assessment. Chest high-resolution computed tomography (HRCT) has become the method of choice to evaluate chronic lung disease, but entails exposure to ionizing radiation. Magnetic resonance imaging (MRI) has been proposed as a potential radiation-free technique in several chest disorders. Aim of our study is to evaluate whether high-field MRI is as effective as HRCT in identifying PCD pulmonary abnormalities. We also analyzed the relationships between the severity and extension of lung disease, and functional data.


METHODS
Thirteen PCD patients (8 children/5 adults; median age, 15.2 yrs) underwent chest HRCT and high-field 3T MRI, spirometry, and deep throat or sputum culture. Images were scored using a modified version of the Helbich system.


RESULTS
HRCT and MRI total scores were 12 (range, 6-20) and 12 (range, 5-17), respectively. Agreement between HRCT and MRI scores was good or excellent (r > 0.8). HRCT and MRI total scores were significantly related to forced vital capacity (r = -0.5, p = 0.05; and r = -0.7, p = 0.009, respectively) and forced expiratory volume at 1 second (r = -0.6, p = 0.03; and r = -0.7, p = 0.009, respectively).


CONCLUSIONS
Chest high-field 3T MRI appears to be as effective as HRCT in assessing the extent and severity of lung abnormalities in PCD. MRI scores might be used for longitudinal assessment and be an outcome surrogate in future studies.
Montella
Silvia
S
Department of Paediatrics, University of Naples Federico II, Naples, Italy. santamar@unina.it.
Santamaria
Francesca
F
Salvatore
Marco
M
Maglione
Marco
M
Iacotucci
Paola
P
De Santi
Maria Margherita
MM
Mollica
Carmine
C