Computed tomography scoring in adults with cystic fibrosis (CF): correlation with clinical and functional measurements

The aim of our study was to evaluate HRCT abnormalities in adult CF patients and to determine correlations between these findings and clinical, exercise, lung function measurements and BMI, if any. 33 patients participated in the study. All patients had documented clinical and genotypic features of CF as well as abnormal sweat test results. CT scans were scored using Bhalla scoring system. Body plethysmography, lung diffusion capacity, oxygen saturation (SpO₂), 6-MWT, dyspnea (by Borgand MRC scale) and BMI were also evaluated. Bronchiectasis were observed in all the patients (30 % mild, 46 % moderate, 24 % severe affecting 1–5 segments in 37 %, 6–9 segments in 37 %, and > 9 segments in 26 %) as well as peribronchial wall thickening (mild in 88 %, moderate in 12 %), with bronchial involvement (up to the 4^th generation in 40 %, up to the 5^th in 37 %, up to the 6^th and more distal in 23 %) and mucus plugging were found in 54 % of patients. Less common abnormalities included sacculations or abscesses, bullae, emphysema and collapse (36, 18, 15 and 15 %, respectively). The overall HRCT score showed significant correlations to lung function, BMI and SpO₂, more closely to bronchiectasis and bronchial involvement. 6-minute distance, though correlating significantly with BMI and lung function, had no correlation with HRCT findings. HRCT is a sensitive tool to evaluate morphologic pulmonary changes in CF patients. Bronchiectasis and peribronchial wall thickening are the most common finding in CF adults. Extent of bronchiectasis and generations of bronchial divisions involved correlated best with clinical, BMI and lung function measurements.

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