Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: HRCT Findings for All Patients

Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: HRCT Findings for All PatientsOn an annual basis, all spirometric variables except RV deteriorated significantly (Table 4). Almost all individual HRCT parameters also deteriorated, with the exception of acinar nodules, mosaic perfusion, and thickening of interlobular septa (Fig 1, bottom, B). Air trapping, collapse/consolidation, mucus plugging, peribronchial thickening, severity and extent of bronchiectasis, generations of bronchial divisions involved, and HRCT total score declined significantly faster than FEV1. FEF25-75 declined at a rate of – 5.1% per year. However, air trapping and extent of bronchiectasis declined significantly faster, at – 5.3 per year and – 6.5% per year, respectively (p < 0.001). Six patients showed stable spirometry results (annual change, 1.4% per year) but worsening HRCT scores (annual change, 6.6% per year). Reading here
HRCT Findings Based on Degree of Lung Impairment
Mucus plugging and extent of bronchiectasis declined at a more significant rate than FEV1 in the group with mildly impaired lung function (Table 4; Fig 2). Air trapping, collapse/consolidation, peribronchial thickening, severity of bronchiectasis, and generations of bronchial divisions involved deteriorated at a more significant rate than FEV1 in the group with moderate-to-severely impaired lung function. The intraclass correlations for HRCT at time 1 and time 2 were 0.83 and 0.80, respectively (p < 0.0001), indicating good interobserver agreement. Interobserver agreement for individual structural abnormalities was poor to moderate. At time 1, the best interobserver agreement was seen for mosaic perfusion (к > 0.8, p < 0.0001), generations of bronchial divisions (к = 0.50, p < 0.0001), air trapping (к = 0.33, p = 0.003), and severity of bronchiectasis (к = 0.25, p = 0.004). At time 2, the best interobserver agreement was seen for mosaic perfusion (к >0.8, p < 0.0001), generations of bronchial divisions (к 0.44, p < 0.0001), collapse/consolidation (к = 0.37, p = 0.001), bullae (к = 0.36, p = 0.02), and air trapping (к = 0.31, p = 0.009).

Table 4—AHRCT and APFT Based on Degree of Impaired Lung Function

Parameters All Patients p Value Mild Lung Impairment p Value Moderate-to-Severe Lung Impairment p Value
FEV1 — 2.3 0.001 — 2.6 0.05 — 2.1 0.002
FVC — 1.4 0.02 — 1.0 0.34 — 1.8 0.02
FEF25-75 — 5.1 0.003 — 7.6 0.0001 — 3.0 0.0001
FEV1/FVC — 1.5 0.002 — 2.5 0.007 — 0.8 0.07
TLC — 2.0 0.04 — 2.4 0.03 — 1.4 0.47
RV 1.4 0.1 — 6.2 0.23 14.0 0.44
Dlco — 4.9 0.001 — 6.5 0.0001 — 3.0 0.2
Air trapping 5.3 0.001 4.8 0.01 5.7 0.02
Collapse/consolidation 3.1 0.1 1.5 0.4 4.2 0.16
Mucous plugging 4.0 0.03 6.6 0.03 2.1 0.2
Peribronchial thickening 4.2 0.005 2.5 0.12 5.3 0.01
Sacculations/abscesses 1.3 0.01 0.5 0.32 1.8 0.3
No. of bullae 0.6 0.01 0.0 0.83 1.1 0.27
Severity of bronchiectasis 4.6 0.0001 1.5 0.47 6.7 0.001
Extent of bronchiectasis 6.5 0.0001 7.6 0.009 5.7 0.002
Ground glass 0.8 0.01 0.5 0.41 1.1 0.32
Emphysema 1.3 0.02 1.5 0.23 1.1 0.54
Acinar nodules — 0.3 0.37 0.8 0.6 — 1.1 0.44
Mosaic perfusion — 0.6 0.37 — 1.5 0.81 0.0 0.31
Thickening of intralobular septa — 0.4 0.1 — 1.5 0.4 0.4 0.56
Generations of bronchial divisions 4.0 0.0001 3.6 0.05 4.2 0.04
Total score 2.7 0.0001 2.3 0.003 3.0 0.002

Figure-2

Figure 2. A 30-year-old woman with CF. Top left, A: Flow-volume (F/V) loop at time 1 demonstrates FEV1 of 2.9 L (94% of predicted). Top right, B: Transverse 1-mm HRCT image through the upper lobes demonstrates moderate bronchiectasis (straight arrow) and peribronchial wall thickening (curved arrow) and an apical bulla (arrowhead). Bottom left, C: Twenty-nine months later, the flow-volume loop demonstrates FEV1 of 2.7 L (88% of predicted). Bottom right, D: Transverse 1-mm HRCT image through the upper lobes demonstrates marked progression of disease with new and severe bilateral upper lobe collapse (note the anterior shift of the left major and right minor fissures, straight arrows) and marked progression in the extent and severity of bronchiectasis (curved arrows).

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