CF derived scoring systems do not fully describe the range of structural changes seen on CT scans in PCD

Abstract

BACKGROUND: Structural lung changes seen on computed tomography (CT) scans in Cystic Fibrosis (CF) and Primary Ciliary Dyskinesia (PCD) are currently described using scoring systems derived from CF populations. This practise assumes lung damage in the two conditions is identical, potentially resulting in a failure to identify PCD-specific changes. Our study addresses this assumption. METHODS: A total of 58 CT scans from 41 PCD patients (age 2-48 years) were examined and the presence and extent of abnormalities common in CF; bronchiectasis, bronchial wall thickening, atelectasis, mucous plugging, and air trapping noted. Further assessment of the PCD scans by an experienced chest radiologist i..