Pulmonary Abnormalities on High-Resolution CT Demonstrate More Rapid Decline Than FEV1 in Adults With Cystic Fibrosis: Results

Mean age of the patients increased by 4 years (Table 2). Mean BMI remained stable, but four patients had BMIs < 18.5 kg/m2 at time 1, indicating malnourishment. Genotype was available for 37 patients. Forty-four percent of patients were homozygous and 36% were heterozygous for the AF508 mutation. All spirometry measurements except RV deteriorated significantly. Mean FEV1 was 81.8 ± 24.9% of predicted at time 1, indicating a group with overall mild lung impairment (Fig 1, top, A). This decreased by 11.7% between PFT-1 and PFT-2 (42 ± 7 months). Sixteen patients had mild lung function abnormalities, with mean FEV1 decreasing from 102.1 ± 14.5 to 87.5 ± 18.2%. Nineteen patients had moderate lung function abnormalities, with mean FEV1 decreasing from 69.8 ± 6.0 to 59.5 ± 15.8%. Four patients had severe lung function abnormalities, with mean FEV1 decreasing from 34.0 ± 2.7 to 30.5 ± 2.6%.

Sputum cultures were available for 28 patients and 34 patients at time 1 and time 2, respectively. The most commonly cultured organism was Pseudomonas aeruginosa (75% of patients at time 1, 74% of patients at time 2). Over the course of the study period, 72% of patients were prescribed pancreatic supplements; 64% were prescribed multivitamins; 23% were prescribed deoxyribonuclease; 64% were prescribed inhaled nebulized antibiotics (56% inhaled colomycin, 8% inhaled tobramycin); 64% were prescribed oral prophylactic antibiotics; 18% were prescribed nasal steroids for sinusitis; 59% were prescribed inhaled (3-agonists; and 33% were prescribed inhaled steroids. The most commonly scored abnormalities were generations of bronchial divisions involved and severity and extent of bronchiectasis, which were seen in > 90% of patients at time 1 and time 2. Bullae were least commonly detected, seen in 3% of patients at time 1 and 8% of patients at time 2. In terms of absolute numbers of patients, of all CT abnormalities air trapping increased the most (31 to 62%, p < 0.001) [Table 3]. Other abnormalities showing a significant absolute increase in numbers of patients included mucus plugging (82 to 90%, p < 0.01), peribronchial thickening (79 to 85%, p < 0.001), and severity and extent of bronchiectasis (90 to 92%, p < 0.01).

Table 2—Patient Demographics, Spirometry Results, and Sputum Culture Findings at Time 1 and Time 2

Variables Time 1 Time 2
Demographics
Age, yr i>+122 26 ± 7
Height, m 1.66 ± 0.1 1.68 ± 0.1
Weight, kg 60.7 ± 11.5 62.4 ± 11.5
BMI, kg/m2 21.9 ± 3.0 21.9 ± 2.8
Genotype, No. of patients!
AF508 homozygous 17
AF508 heterozygous 13
Other 7
Spirometry, % predicted
FEV: (n = 39) 81.8 ± 24.9 70.2 ± 24.9
FVC (n = 39) 91.9 ± 17.5 83.0 ± 19.5
FEF25-75 (n = 28) 56.8 ± 38.0 40.8 ± 33.4
FEV1/FVC 74.7 ± 14.1 70.4 ± 12.8
RV (n = 16) 113.3 ± 37.7 114.3 ± 67.3
TLC (n = 16) 99.3 ± 15.3 93.5 ± 16.5
RV/TLC 121.1 ± 25.9 117.7 ± 39
Dlco (n = 21) 98.4 ± 14.7 89.0 ± 15.4
Sputum culture findings, % of patientsj
P aeruginosa 75 74
Staphylococcus aureus 39 47
Aspergillus fumigatus 14 12
Streptococcus pneumoniae 7 0
Haemophilus influenzae 4 3
Burkholderia cepecia 0 3
Escherichia coli 0 3
Pseudomonas mirabilis 7 9
Candida sp 55 34
Other 14 15

Table 3—Longitudinal Changes in Absolute Number of Patients With HRCT Abnormalities

HRCT Abnormalities Time 1 Time 2 Difference
Air trapping 12 (31) 24 (62) 12.0 (30.8)
Collapse/consolidation 15 (38) 19 (49) 4.0 (10.3)
Mucous plugging 32 (82) 35 (90) 3.0 (7.7)
Peribronchial thickening 31 (79) 33 (85) &СCN
Sacculations/abscesses 5(13) 7(18) 2.0 (5.1)
No. of bullae 1(3) 3 (8) 2.0 (5.1)
Severity of bronchiectasis 35 (90) 36 (92) 1.0 (2.6)
Extent of bronchiectasis 35 (90) 36 (92) 1.0 (2.6)
Ground glass 3 (8) 4 (10) 1.0 (2.6)
Emphysema 2 (5) 3 (8) 1.0 (2.6)
Acinar nodules 15 (38) 15 (38) 0.0 (0)
Mosaic perfusion 11 (28) 10 (26) — 1.0 (-2.6)
Thickening of intralobular septa 7 (18) 6(15) — 1.0 (-2.6)
Generations of bronchial divisions 36 (92) 36 (92) 0(0)

Figure-1

Figure 1. Changes in lung function impairment and composite HRCT scores in 39 adults with CF over a mean 4-year period. Overall FEV1 deteriorated by 2.3% per year (top, A) while composite HRCT scores deteriorated by 2.7% per year (bottom, B).

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