Imagerie thoracique de la dyskinésie ciliaire primitive

Résumé 0

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.