Initial clinical features and cystic fibrosis(CF) genotype of the study patients
| Subjects n | 144 |
| Age yrs | 25±5.9 (17–43) |
| Sex ratio | 1.03 |
| Male | 73 (50.7) |
| Female | 71 (49.3) |
| Past medical history of autoimmune disease | 4 (2.8) |
| Past medical history of drug hypersensitivity | 50 (34.7) |
| CFTR gene mutation | |
| F508del/F508del | 57 (39.6) |
| Others | 87 (60.4) |
| Pulmonary involvement | 143 (99.3) |
| Chronic exocrine pancreatic insufficiency | 134 (93.1) |
| Chronic sinusitis | 78 (54.2) |
| BMI ≤19 kg·m−2 | 67 (46.5) |
| BMI kg·m−2 | 19.57±2.8 |
| Chronic diarrhoea | 51 (35.4) |
| CF-related liver disease | 46 (31.9) |
| Diabetes mellitus | 34 (23.6) |
| Arthralgia | 13 (9) |
| Associated with polyarthritis | 2 (1.3) |
| Associated with oligo/monarthritis | 3 (2.1) |
| FEV1 % pred | 52.2±21.9 |
| FEV1 >75% pred | 23 (16) |
| 75%≥FEV1≥30% pred | 90 (65.2) |
| FEV1 <30% pred | 31 (21.5) |
| Reversible airway obstruction with β2-agonists | 37 (25.7) |
| Antibiotic courses for pulmonary exacerbation in past 12 months# | 117 (81.3) |
| 0 | 27 (18.7) |
| 1 | 35 (24.3) |
| 2 | 26 (18.1) |
| 3 | 36 (25) |
| ≥4 | 20 (13.9) |
Data are presented as mean±sd (range), n (%) or mean±sd, unless otherwise stated. CFTR: CF transmembrane conductance regulator gene; BMI: body mass index; FEV1: forced expiratory volume in 1 s; % pred: % predicted. #: mean number of courses = 1.6.