Pulmonary abnormalities on high-resolution CT demonstrate more rapid decline than FEV1 in adults with cystic fibrosis.

BACKGROUND: FEV1 may remain stable while high-resolution CT (HRCT) appearances deteriorate in children with cystic fibrosis (CF). However, spirometry results commonly decline in older age groups. OBJECTIVES: To compare the rate of decline in HRCT abnormalities and spirometry results over time in an adult cohort with CF. METHODS: The HRCT scans of 39 consecutive patients (19 males and 20 females; mean age, 22 years; range, 16 to 48 years) with two HRCT scans > 18 months apart were randomly and blindly scored using a modified Bhalla scoring system by two independent chest radiologists. Age, body mass index, spirometry, and sputum cultures were recorded at the time of both HRCTs. Rates of change in clinical parameters and HRCT abnormalities were calculated and compared using repeated-measures analysis of variance. RESULTS: Mean FEV1 declined at a rate of - 2.3% per year, while mean HRCT total score declined at a rate of -2.7% per year. Several individual HRCT abnormalities as well as HRCT total scores declined significantly faster than FEV1 (p < 0.001). Six patients showed stable spirometry results but worsening HRCT scores. Mucus plugging and extent of bronchiectasis deteriorated at a more rapid rate in the group with mildly impaired lung function. Air trapping, collapse/consolidation, peribronchial thickening, severity of bronchiectasis, and generations of bronchial divisions involved deteriorated at a more rapid rate in the group with moderate-to-severely impaired lung function. CONCLUSIONS: Adult CF patients have more rapid rates of decline in HRCT abnormalities than in spirometry results. Individual HRCT abnormalities decline at different rates depending on the degree of lung function impairment.

Hyperpolarized 3helium magnetic resonance ventilation imaging of the lung in cystic fibrosis: comparison with high resolution CT and spirometry.

The purpose of this study was to compare hyperpolarized 3helium magnetic resonance imaging (3He MRI) of the lungs in adults with cystic fibrosis (CF) with high-resolution computed tomography (HRCT) and spirometry. Eight patients with stable CF prospectively underwent 3He MRI, HRCT, and spirometry within 1 week. Three-dimensional (3D) gradient-echo sequence was used during an 18-s breath-hold following inhalation of hyperpolarized 3He. Each lung was divided into six zones; 3He MRI was scored as percentage ventilation per lung zone. HRCT was scored using a modified Bhalla scoring system. Univariate (Spearman rank) and multivariate correlations were performed between 3He MRI, HRCT, and spirometry. Results are expressed as mean+/-SD (range). Spirometry is expressed as percent predicted. There were four men and four women, mean age = 31.9+/-9 (20-46). Mean forced expiratory volume in 1 s (FEV)1 = 52%+/-29 (27-93). Mean 3He MRI score = 74%+/-25 (55-100). Mean HRCT score = 48.8+/-24 (13.5-83). The correlation between 3He MRI and HRCT was strong (R = +/-0.89, p < 0.001). Bronchiectasis was the only independent predictor of 3He MRI; 3He MRI correlated better with FEV1 and forced vital capacity (FVC) (R = 0.86 and 0.93, p < 0.01, respectively) than HRCT (R = +/-0.72 and +/-0.81, p < 0.05, respectively). This study showed that 3He MRI correlates strongly with structural HRCT abnormalities and is a stronger correlate of spirometry than HRCT in CF.

Thin-section CT in patients with cystic fibrosis: correlation with peak exercise capacity and body mass index.

PURPOSE: To evaluate whether thin-section chest computed tomographic (CT) findings correlate with exercise capacity, body mass index (BMI), dyspnea, and leg discomfort in patients with cystic fibrosis (CF). MATERIALS AND METHODS: Institutional ethics committee approval was obtained, and patients provided written informed consent. Twenty-two patients (13 male and nine female patients; mean age, 22 years +/- 5.9; age range, 17-41 years) with stable CF underwent thin-section CT and exercise testing on a cycle ergometer. Three radiologists blinded to the clinical severity of disease and the spirometric findings of all patients independently and randomly scored all scans with a modified Bhalla scoring system. The primary measurement of the outcome of exercise testing was percentage of predicted peak O2 uptake. Univariate (Spearman rank correlation) and multivariate analyses were used to compare thin-section CT, clinical (age, sex, spirometric data, and BMI), and exercise measurements. RESULTS: The correlation between total thin-section CT score and percentage of predicted peak O2 uptake was stronger than the correlation between the percentage of predicted peak O2 uptake and any clinical measurement (R = -0.60, P < .01). The thin-section CT structural abnormalities that had the strongest correlation with percentage of predicted peak O2 uptake were severity of bronchiectasis and presence of sacculations or abscesses (R = -0.70 and -0.71, respectively; P < .01). Multivariate analysis showed total thin-section CT score to be the only significant predictor of exercise capacity, accounting for 42% of the variance in percentage of predicted peak O2 uptake. CONCLUSION: In patients with CF, the correlation between thin-section CT score and exercise limitation is stronger than that between spirometry results or BMI and exercise limitation.