Quantitative Evaluation of High-Resolution CT Findings in Advanced Cystic Fibrosis Patients Based on the Brody Scoring

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Background: Progressive destruction of the lungs leads to increased morbidity and mortality rates in patients with CF. In various studies, it has been shown that quantitative evaluation of parenchymal changes in lung high-resolution computed tomography (HRCT) in CF patients measured by the 2006 revised Brody scoring is compatible with the clinical status. CF patients are mostly children who cannot perform complicated pulmonary tests. Unfortunately, screening tests are not performed in many countries, such as Iran, resulting in late diagnosis in older ages, with consequently more diffuse parenchymal involvement and more hospitalization. The quantitative measurement of parenchymal changes in HRCT seems to be useful for early and accurate evaluation of the clinical status. Objectives: The aim of this study was to determine the severity and prevalence of pulmonary changes in late diagnosed CF patients by means of the HRCT Brody scoring system. Patients and Methods: Pulmonary HRCT results of 23 CF patients with the mean age of 13.5 years (5 - 18 years) were scored with the Brody scoring system. The system provided sub-scores for evaluating the presence and severity of five variables in central and peripheral regions of six lung lobes. Results: All studied patients had bronchiectasis and peribronchial wall thickening (PBWT) which were caused by the disease chronicity. However, parenchymal changes in the form of ground glass opacity (GG) and consolidation (CON) were significantly less prevalent, compared to other parameters. The highest score among all lobes was related to bronchiectasis and the lowest to the parenchymal changes (CON, GG). The scoring parameters of bronchiectasis, PBWT, GG, CON, and air trapping showed no significant difference in lobar, central or peripheral involvement, while only mucous plugging was more prominent in the peripheral zones. The contribution of lobar involvement appeared in the following order: right upper lobe (RUL) > left lower lobe (LLL) > right lower lobe (RLL) > left upper lobe (LUL) > Lingula, right middle lobe (RML). Conclusions: It seems that in advanced cases of CF, at older ages and with lack of appropriate medical care, severity, prevalence, and appearance of lung lesions do not follow a definite specific pattern. Therefore, a similar study is suggested in older patients with a larger sample size for comparison of results.