Table 2.
Patient characteristics in IPF patients subdivided according to MUC5B rs35705950 and DSP rs2076295 genotypes.
| N (%)/valid N, median (5th; 95th percentile) | Total | MUC5B rs35705950 | p a | DSP rs2076295 | p a | ||
|---|---|---|---|---|---|---|---|
| Carriage T | Genotype GG | Carriage G | Genotype TT | ||||
| N = 210 | n = 116 | n = 94 | n = 123 | n = 87 | |||
| Sex | |||||||
| Female | 71 (33.8%) | 29 (25.0%) | 42 (44.7%) | 0.003 | 40 (32.5%) | 31 (35.6%) | 0.639 |
| Male | 139 (66.2%) | 87 (75.0%) | 52 (55.3%) | 83 (67.5%) | 56 (64.4%) | ||
| Age at the time of diagnosis | 210, 68 (49;80) | 116, 68 (51;81) | 94, 66 (48;80) | 0.137 | 123, 68 (50;81) | 87, 68 (49;77) | 0.568 |
| Duration of symptoms | 210, 12.0 (3.0;48.0) | 116, 12.0 (3.0;48.0) | 94, 11.0 (3.0;48.0) | 0.596 | 123, 12.0 (3.0;36.0) | 87, 12.0 (3.0;48.0) | 0.172 |
| Finger clubbing | 102 (48.6%) | 54 (46.6%) | 48 (51.1%) | 0.515 | 63 (51.2%) | 39 (44.8%) | 0.361 |
| Cough | 146 (73.7%) | 77 (71.3%) | 69 (76.7%) | 0.391 | 84 (73.0%) | 62 (74.7%) | 0.794 |
| Exertional dyspnoea | |||||||
| 1 | 2 (1.0%) | 2 (1.8%) | 0 (0.0%) | 0.118 | 2 (1.7%) | 0 (0.0%) | 0.323 |
| 2 | 134 (67.0%) | 79 (70.5%) | 55 (62.5%) | 78 (67.2%) | 56 (66.7%) | ||
| 3 | 64 (32.0%) | 31 (27.7%) | 33 (37.5%) | 36 (31.0%) | 28 (33.3%) | ||
| FVC | |||||||
| Diagnosis | 165, 73 (50;101) | 90, 74 (50;105) | 75, 71 (48;99) | 0.405 | 98, 72 (48;98) | 67, 76 (50;105) | 0.271 |
| 6 months | 126, 78 (43;104) | 67, 78 (43;104) | 59, 78 (42;104) | 0.655 | 77, 76 (41;104) | 49, 81 (48;104) | 0.110 |
| Diff. diag. – 6 months | 122, -0.80 (–15.22;12.66) | 65, 0.19 (–13.86;12.17) | 57, –2.50 (–22.21;17.61) | 0.163 | 73, –0.68 (–15.22;12.66) | 49, –0.95 (–15.65;13.07) | 0.605 |
| With progression (decline ⩾ 10%) | 12 (9.8%) | 7 (10.8%) | 5 (8.8%) | 0.711 | 6 (8.2%) | 6 (12.2%) | 0.468 |
| 12 months | 125, 76 (44;102) | 70, 77 (43;104) | 55, 73 (44;102) | 0.872 | 72, 76 (41;102) | 53, 76 (48;104) | 0.487 |
| Diff. diag. – 12 months | 121, 0.68 (–13.38;16.10) | 67, 0.99 (–11.55;17.11) | 54, 0.10 (–24.74;12.03) | 0.301 | 68, –0.59 (–19.54;12.31) | 53, 1.46 (–11.93;17.28) | 0.426 |
| With progression (decline ⩾ 10%) | 19 (15.7%) | 13 (19.4%) | 6 (11.1%) | 0.207 | 8 (11.8%) | 11 (20.8%) | 0.179 |
| 18 months | 102, 76 (42;107) | 57, 78 (40;110) | 45, 73 (42;97) | 0.226 | 63, 76 (40;97) | 39, 77 (43;110) | 0.326 |
| Diff. diag. – 18 months | 97, 2.35 (–14.67;16.91) | 54, 1.36 (–16.56;16.91) | 43, 3.87 (–10.50;16.30) | 0.085 | 58, 2.39 (–14.67;16.30) | 39, 1.62 (–16.56;19.20) | 0.930 |
| With progression (decline ⩾ 10%) | 12 (12.4%) | 4 (7.4%) | 8 (18.6%) | 0.096 | 5 (8.6%) | 7 (17.9%) | 0.176 |
| DLCO | |||||||
| Diagnosis | 165, 46.9 (29.0;72.3) | 90, 47.5 (29.0;75.0) | 75, 45.2 (29.0;69.7) | 0.624 | 98, 47.0 (29.0;72.3) | 67, 46.0 (33.2;73.7) | 0.708 |
| 6 months | 126, 47.9 (25.5;77.9) | 67, 48.3 (27.0;77.9) | 59, 46.7 (7.3;78.2) | 0.936 | 77, 48.1 (13.5;77.9) | 49, 47.0 (29.2;83.7) | 0.736 |
| Diff. diag. – 6 months | 122, 1.74 (–12.73;18.30) | 65, 2.54 (–10.51;16.87) | 57, 1.21 (–14.12;26.22) | 0.693 | 73, 0.92 (–16.38;19.86) | 49, 2.83 (–10.02;13.43) | 0.258 |
| With progression (decline ⩾ 15%) | 11 (9.0%) | 6 (9.2%) | 5 (8.8%) | 0.930 | 9 (12.3%) | 2 (4.1%) | 0.102 |
| 12 months | 124, 47.1 (23.8;68.9) | 69, 47.2 (22.4;68.9) | 55, 47.1 (25.2;71.6) | 0.898 | 72, 47.7 (22.4;75.8) | 52, 45.7 (25.8;66.9) | 0.693 |
| Diff. diag. – 12 months | 120, 3.23 (–13.07;19.91) | 66, 1.04 (–12.90;22.36) | 54, 3.55 (–13.50;15.55) | 0.752 | 68, 3.04 (–13.50;17.71) | 52, 3.55 (–12.19;20.34) | 0.395 |
| With progression (decline ⩾ 15%) | 17 (14.2%) | 13 (19.7%) | 4 (7.4%) | 0.048 | 10 (14.7%) | 7 (13.5%) | 0.846 |
| 18 months | 100, 45.3 (17.8;67.9) | 55, 49.9 (26.8;68.4) | 45, 39.6 (14.8;66.2) | 0.005 | 63, 43.2 (17.8;66.2) | 37, 48.8 (20.6;75.5) | 0.178 |
| Diff. diag. – 18 months | 95, 2.81 (–11.27;28.33) | 52, 0.32 (–11.27;28.33) | 43, 4.32 (–8.78;27.06) | 0.098 | 58, 2.86 (–9.83;41.89) | 37, 2.17 (–12.62;28.33) | 0.174 |
| With progression (decline ⩾ 15%) | 13 (13.7%) | 4 (7.7%) | 9 (20.9%) | 0.061 | 7 (12.1%) | 6 (16.2%) | 0.569 |
| Patients with exacerbation (yes) | 49 (23.3%) | 27 (23.3%) | 22 (23.4%) | 0.983 | 26 (21.1%) | 23 (26.4%) | 0.373 |
| Number of exacerbations | |||||||
| 1 | 33 (67.3%) | 18 (66.7%) | 15 (68.2%) | 0.262 | 17 (65.4%) | 16 (69.6%) | 0.727 |
| 2 | 13 (26.5%) | 7 (25.9%) | 6 (27.3%) | 7 (26.9%) | 6 (26.1%) | ||
| 3 | 2 (4.1%) | 2 (7.4%) | 0 (0.0%) | 1 (3.8%) | 1 (4.3%) | ||
| 4 | 1 (2.0%) | 0 (0.0%) | 1 (4.5%) | 1 (3.8%) | 0 (0.0%) | ||
| Histopathological finding | |||||||
| UIP | 58 (69.9%) | 33 (75.0%) | 25 (64.1%) | 0.719 | 28 (70.0%) | 30 (69.8%) | 0.983 |
| Probable UIP | 12 (14.5%) | 5 (11.4%) | 7 (17.9%) | 6 (15.0%) | 6 (14.0%) | ||
| Indeterminate for UIP | 5 (6.0%) | 2 (4.5%) | 3 (7.7%) | 2 (5.0%) | 3 (7.0%) | ||
| Inconsistent with UIP | 8 (9.6%) | 4 (9.1%) | 4 (10.3%) | 4 (10.0%) | 4 (9.3%) | ||
| Radiological pattern. HRCT pattern | |||||||
| UIP pattern | 171 (81.4%) | 96 (82.8%) | 75 (79.8%) | 0.262 | 99 (80.5%) | 72 (82.8%) | 0.064 |
| Probable UIP | 34 (16.2%) | 19 (16.4%) | 15 (16.0%) | 19 (15.4%) | 15 (17.2%) | ||
| Indeterminate for UIP | 5 (2.4%) | 1 (0.9%) | 4 (4.3%) | 5 (4.1%) | 0 (0.0%) | ||
| HRCT alveolar score | |||||||
| 0 | 65 (41.4%) | 36 (42.4%) | 29 (40.3%) | 0.783 | 43 (45.3%) | 22 (35.5%) | 0.249 |
| 1 | 42 (26.8%) | 22 (25.9%) | 20 (27.8%) | 20 (21.1%) | 22 (35.5%) | ||
| 2 | 43 (27.4%) | 24 (28.2%) | 19 (26.4%) | 28 (29.5%) | 15 (24.2%) | ||
| 3 | 6 (3.8%) | 3 (3.5%) | 3 (4.2%) | 3 (3.2%) | 3 (4.8%) | ||
| 4 | 1 (0.6%) | 0 (0.0%) | 1 (1.4%) | 1 (1.1%) | 0 (0.0%) | ||
| HRCT interstitial score | |||||||
| 1 | 15 (9.6%) | 11 (12.9%) | 4 (5.6%) | 0.319 | 7 (7.4%) | 8 (12.9%) | 0.002 |
| 2 | 69 (43.9%) | 35 (41.2%) | 34 (47.2%) | 42 (44.2%) | 27 (43.5%) | ||
| 3 | 53 (33.8%) | 30 (35.3%) | 23 (31.9%) | 28 (29.5%) | 25 (40.3%) | ||
| 4 | 19 (12.1%) | 9 (10.6%) | 10 (13.9%) | 18 (18.9%) | 1 (1.6%) | ||
| 5 | 1 (0.6%) | 0 (0.0%) | 1 (1.4%) | 0 (0.0%) | 1 (1.6%) | ||
| Neutrophil granulocyte (%) | |||||||
| Neutrophil granulocyte > 3% | 173, 7.00 (0.00;30.00) | 96, 7.00 (0.00;30.00) | 77, 5.00 (0.00;47.00) | 0.055 | 103, 5.00 (0.00;25.00) | 70, 7.00 (1.00;51.00) | 0.157 |
| 121 (69.9%) | 75 (78.1%) | 46 (59.7%) | 0.009 | 70 (68.0%) | 51 (72.9%) | 0.489 | |
| Lymphocytes (%) | |||||||
| Lymphocytes > 15% | 174, 8.00 (1.00;32.00) | 95, 8.00 (1.00;29.00) | 79, 7.00 (1.60;38.00) | 0.817 | 104, 8.00 (1.00;27.70) | 70, 7.00 (2.00;38.00) | 0.567 |
| 34 (19.5%) | 19 (20.0%) | 15 (19.0%) | 0.867 | 20 (19.2%) | 14 (20.0%) | 0.900 | |
| Smoking | |||||||
| Ex-smoker | 100 (47.6%) | 56 (48.3%) | 44 (46.8%) | 0.970 | 61 (49.6%) | 39 (44.8%) | 0.780 |
| Smoker | 2 (1.0%) | 1 (0.9%) | 1 (1.1%) | 1 (0.8%) | 1 (1.1%) | ||
| Nonsmoker | 108 (51.4%) | 59 (50.9%) | 49 (52.1%) | 61 (49.6%) | 47 (54.0%) | ||
| Medication | |||||||
| Pirfenidone | 40 (19.0%) | 27 (23.3%) | 13 (13.8%) | 0.212 | 24 (19.5%) | 16 (18.4%) | 0.923 |
| Nintedanib | 127 (60.5%) | 66 (56.9%) | 61 (64.9%) | 73 (59.3%) | 54 (62.1%) | ||
| Non-IPF medication | 43 (20.5%) | 23 (19.8%) | 20 (21.3%) | 26 (21.1%) | 17 (19.5%) | ||
| Therapeutic response | 22 (10.5%) | 10 (8.6%) | 12 (12.8%) | 0.331 | 12 (9.8%) | 10 (11.5%) | 0.686 |
Diff. diag., the difference between the FVC or DLCO at diagnosis and given timepoint; DLCO, diffuse lung capacity; FVC, forced vital capacity; HRCT, high-resolution computed tomography; IPF, idiopathic pulmonary fibrosis; UIP, usual interstitial pneumonia.
a
Statistical significance was tested by chi-square test for categorical variables and by Kruskal–Wallis test for continuous variables.