The Relationship of Radiological Scoring with Clinical Findings and Pulmonary Function Decline in Primary Ciliary Dyskinesia

Abstract

<b>Introduction and objective:</b> Primary ciliary dyskinesia(PCD) is a genetic disease characterized with recurrent lung infections and bronchiectasis. The primary aim of this study is to analyze the relationship between radiological scoring and clinical, laboratory findings of PCD patients. The secondary aim is to evaluate the predictive role of radiological scoring on pulmonary function decline. <b>Method:</b> In this cross-sectional study, the relationship between chest computed tomography(CT) findings, pulmonary function tests, microbiological examinations and clinical symptoms of 117 patients with PCD were evaluated. Chest CT were evaluated according to the “Brody” score. <b>Results:</b> On chest CT, the median(25-75p) total bronchiectasis score was 12(6-16.8); peribronchial thickening score was 11(5.1-16) and mucus plugging score was 5(2-9). There was no significant correlation between the patients9 age at diagnosis, gender, PICADAR scores and chest CT scores. The total bronchiectasis, mucus plugging, peribronchial thickening, parenchyma and hyperinflation scores were higher in patients with CCNO mutations compared to other mutations, which was not statistically significant. There was low grade significant negative correlation between total bronchiectasis scores and FEV1(r:-0.47), FEF25-75(r:-0.42), FVC(r:-0.35), FEV1/FVC(r:-0.29) z-scores. Increased hyperinflation and right lower lobe score affected the FEV1 decline 1.1 times(95%CI: 1.02-1.39, 95%CI: 1-1.2 respectively). <b>Conclusion:</b> In our study, we showed that high hyperinflation score, which is an indicator of airway obstruction, is a parameter that predicts FEV1 decline and attention should be paid in the follow-up of patients.