Table 1.
Study population and results from immunophenotyping measurements.
| 0 (n=77) | T1 (n=77) | T2 (n=77) | p-value (T0-T1) | p-value (T1-T2) | p-value (T0-T2) | |
|---|---|---|---|---|---|---|
| Age, years | 33.77 ± 11.52 | |||||
| Female sex, n (%) | 34 (44.2) | |||||
| ppFEV1 | 44.86 ± 20.27 | 57.37 ± 22.33 | 56.92 ± 22.11 | <0.001 | 0.261 | <0.001 |
| BMI, kg/m2 | 20.09 ± 2.58 | 21.13 ± 2.69 | 21.64 ± 2.63 | <0.001 | <0.001 | <0.001 |
| Six-minute walk test, m | 493.4 ± 104.7 | 578.1 ± 118.6 | 589.2 ± 114.7 | <0.001 | 0.079 | <0.001 |
| Sweat chloride, mmol/L | 105.52 ± 12.16 | 56.37 ± 19.06 | 52.24 ± 17.97 | <0.001 | 0.083 | <0.001 |
| Leukocytes,/nL | 10.60 ± 3.50 | 7.34 ± 2.49 | 7.75 ± 2.63 | <0.001 | 0.046 | <0.001 |
| CRP, mg/dL | 1.25 ± 1.81 | 0.02 ± 0.13 | 0.05 ± 0.25 | <0.001 | 0.864 | <0.001 |
| P. aeruginosa infection, n (%) | ||||||
| Chronic | 37 (48.1) | |||||
| Non chronic | 40 (52.0) | |||||
| CFTR genotype, n (%) | ||||||
| Homozygous dF508 | 40 (52.0) | |||||
| Heterozygous dF508 | 34 (44.2) | |||||
| Other* | 3 (3.9) | |||||
| Prior CFTR modulator therapy, n (%) | ||||||
| Tezacaftor/Ivacaftor | 34 (44.2) | |||||
| Lumacaftor/Ivacaftor | 3 (3.9) | |||||
| Ivacaftor | 0 (0.0) | |||||
| None | 40 (52.0) | |||||
| Immunophenotyping | ||||||
| CD3+ T cells, % PBMC | 42.20 ± 16.65 | 42.58 ± 15.35 | 45.23 ± 13.81 | 0.831 | 0.126 | 0.128 |
| CD8+ T cells, % T cells | 24.50 ± 8.52 | 26.10 ± 7.67 | 25.48 ± 6.90 | 0.037 | 0.240 | 0.168 |
| CD4+ T helper, % T cells | 63.65 ± 10.40 | 62.29 ± 10.35 | 63.16 ± 8.99 | 0.121 | 0.279 | 0.602 |
| CD25+CD127- Treg, % Th | 8.30 ± 2.37 | 9.85 ± 3.18 | 9.70 ± 2.75 | <0.001 | 0.645 | <0.001 |
| CD39+ Treg, % Treg | 45.16 ± 14.06 | 51.68 ± 12.72 | 51.84 ± 11.41 | <0.001 | 0.895 | <0.001 |
| FoxP3+ Treg, % Treg** | 82.39 ± 6.99 | 79.86 ± 10.38 | 0.221 | |||
| Effector Th, % Th | 87.90 ± 3.11 | 86.69 ± 4.20 | 86.56 ± 3.70 | 0.011 | 0.781 | 0.001 |
| Th1, % effector Th | 10.02 ± 4.90 | 10.23 ± 4.39 | 11.15 ± 4.97 | 0.735 | 0.133 | 0.063 |
| Th2, % effector Th | 68.07 ± 13.14 | 67.60 ± 13.61 | 65.54 ± 14.21 | 0.648 | 0.106 | 0.221 |
| Th17, % effector Th | 10.14 ± 4.22 | 10.12 ± 4.39 | 9.47 ± 4.15 | 0.975 | 0.147 | 0.150 |
| Th1-17, % effector Th | 7.72 ± 4.57 | 7.80 ± 4.41 | 8.91 ± 5.47 | 0.792 | 0.071 | 0.087 |
Values are mean ± standard deviation. p-values were obtained using Wilcoxon test or t test for paired samples. BMI, body mass index; CFTR, cystic fibrosis transmembrane conductance regulator; CRP, C-reactive protein; PBMC, peripheral blood mononuclear cell; ppFEV1, percent predicted forced expiratory volume in 1 second; Th, helper T cells; Treg, regulatory T cells. *Other: R553X/I336K, G542X/3849+10KbC->T, R1162X/A455E. **FoxP3 expression determined in stored samples from a subcohort of 10 PwCF.
Characteristics at baseline (T0), at 3 months (T1) and at 6 months (T2).
Bold values denote statistical significance.