Objectives : Few studies have been published on pulmonary imaging in PCD with high
resolution computed tomography (HRCT). We retrospectively examined HRCT characteristics
and correlations with clinical features in a series of patients with PCD.
Materials and methods : cases were recruited through a Swiss national registry for rare lung
diseases. Clinical data were retrieved by questionnaires to the caring physicians. HRCT were
centrally reviewed and scored separately by 2 expert radiologists unaware of clinical data.
Discrepancies were resolved by a second consensus reading.
Results : 33 patients were included. Bronchiectasis were present in 85%. The middle lobe
was the most frequently affected, followed by the lower lobes, whereas the upper lobes
were relatively spared. Lobar atelectasis was found in 24% and affected only the middle lobe.
No significant correlations were found between a bronchiectasis severity score and,
respectively, clinical characteristics and lung function tests. Signs of bronchiolitis were
common and followed the same spatial distribution as bronchiectasis.
Conclusion : the middle and lower lobes are the most frequently and severely affected by
bronchiectasis in PCD patients. Signs of bronchiolitis are frequently found and co-localize
with bronchiectasis.