Abstract
ObjectivesAmong patients with non-cystic fibrosis bronchiectasis, 1–18% have an underlying diagnosis of primary ciliary dyskinesia (PCD) and it is suspected that there is under-recognition of this disease. Our intention was to evaluate the specific features of PCD seen on computed tomography (CT) in the cohort of bronchiectasis in order to facilitate the diagnosis.Materials and methodsOne hundred and twenty-one CTs performed in patients with bronchiectasis were scored for the involvement, type, and lobar distribution of bronchiectasis, bronchial dilatation, and bronchial wall thickening. Later, associated findings such as mucus plugging, tree in bud, consolidations, ground glass opacities, interlobular thickening, intralobular lines, situs inversus, emphysema, mosaic attenuation, and atelectasis were registered. Patients with PCD (n = 46) were compared to patients with other underlying diseases (n = 75).ResultsIn patients with PCD, the extent and severity of the bronchiectasis and bronchial wall thickness were significantly lower in the upper lung lobes (p<0.001-p = 0.011). The lobar distribution differed significantly with a predominance in the middle and lower lobes in patients with PCD (<0.001). Significantly more common in patients with PCD were mucous plugging (p = 0.001), tree in bud (p <0.001), atelectasis (p = 0.009), and a history of resection of a middle or lower lobe (p = 0.047). Less common were emphysematous (p = 0.003) and fibrotic (p<0.001) changes. A situs inversus (Kartagener’s Syndrome) was only seen in patients with PCD (17%, p <0.001).ConclusionTypical imaging features in PCD include a predominance of bronchiectasis in the middle and lower lobes, severe tree in bud pattern, mucous plugging, and atelectasis. These findings may help practitioners to identify patients with bronchiectasis in whom further work-up for PCD is called for.
Highlights
Bronchiectasis is a condition which has permanent dilation of the bronchi and bronchioles as a result of the destruction of the bronchial wall and elastic connective tissue
In patients with primary ciliary dyskinesia (PCD), the extent and severity of the bronchiectasis and bronchial wall thickness were significantly lower in the upper lung lobes (p
Patients who fulfilled the following diagnostic criteria had a diagnosis of definite PCD: 1) clinical presentation consistent with PCD and 2) consistent findings specific for PCD according to at least two methods, i.e. high-frequency video microscopy analysis (HVMA), transmission electronic microscopy (TEM), immunofluorescence microscopy (IF), nNO (
Summary
Bronchiectasis is a condition which has permanent dilation of the bronchi and bronchioles as a result of the destruction of the bronchial wall and elastic connective tissue. There are various aetiologies which may result in bronchiectasis, including postinfective, chronic obstructive pulmonary disease, connective tissue disease, immunodeficiency, and inherited disorders such as primary ciliary dyskinesia (PCD) [2]. PCD is an autosomal, recessive, inherited disorder resulting in ultrastructural defects of the ciliary apparatus with consequent abnormal or absent beating of cilia [3]. This interferes with normal mucociliary clearance and causes repeated respiratory infections leading to airway damage and increasing the risk for the development of bronchiectasis [4]. Among patients with non-cystic fibrosis bronchiectasis, 1–18% have an underlying diagnosis of inherited disorders including PCD, and it is suspected that there is insufficient recognition of this disease [5,6,7]
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