Characteristics of 1572 adults with cystic fibrosis (CF) according to the seven subgroups identified by cluster analysis
| Cluster 1 | Cluster 2 | Cluster 3 | Cluster 4 | Cluster 5 | Cluster 6 | Cluster 7 | |
| Subjects | 262 | 351 | 674 | 72 | 125 | 37 | 51 |
| Male | 65 (170) | 50 (176) | 49 (328) | 57 (41) | 62 (78) | 62 (23) | 55 (28) |
| Age years | 21.8 (19.9–26.4) | 30.2 (23.7–40.4) | 24.4 (20.7–29.9) | 24.5 (20.3–33.1) | 25.2 (22.3–29.9) | 23.5 (21.7–27.8) | 25.5 (21.0–33.3) |
| Age at diagnosis years# | 1.1 (0.1–7.5) | 17.4 (4.3–31.4) | 0.7 (0.1–3.8) | 1.9 (0.1–11.7) | 0.7 (0.2–2.7) | 0.9 (0.2–5.6) | 0.9 (0.2–5.6) |
| CFTR genotype | |||||||
| Class I–III/class I–III | 78.2 (205) | 4.6 (16) | 79.4 (535) | 69.4 (50) | 75.2 (94) | 75.7 (28) | 64.7 (33) |
| At least one class IV or V mutation | 0.4 (1) | 44.7 (157) | 1.5 (10) | 12.5 (9) | 3.2 (4) | 2.7 (1) | 9.8 (5) |
| Other mutation combinations¶ | 12.2 (32) | 17.3 (61) | 10.5 (71) | 9.7 (7) | 12.0 (15) | 18.7 (7) | 15.7 (8) |
| Incomplete genotype | 9.2 (24) | 33.3 (117) | 8.6 (58) | 8.3 (6) | 9.6 (12) | 2.7 (1) | 9.8 (5) |
| FEV1 L | 2.63 (1.85–3.30) | 2.35 (1.57–3.24) | 1.87 (1.32–2.56) | 1.55 (1.14–2.44) | 0.94 (0.73–1.21) | 1.61 (1.24–2.44) | 1.65 (1.19–2.38) |
| FEV1 % pred | 71.8 (50.5–88.1) | 70.8 (48.2–92.7) | 54.4 (38.9–73.8) | 41.9 (33.6–66.2) | 25.8 (20.8–32.2) | 46.1 (33.3–67.4) | 48.9 (33.3–67.8) |
| Pancreatic insufficiency | 93.1 (244) | 37.6 (132) | 97.0 (654) | 86.1 (62) | 96.0 (120) | 94.6 (35) | 86.3 (44) |
| BMI kg·m−2 | 20.3 (19.0–21.8) | 21.5 (19.7–23.7) | 19.5 (18.0–21.0) | 18.8 (17.2–20.9) | 18.0 (16.7–19.6) | 19.0 (17.7–21.2) | 20.0 (17.9–22.8) |
| Airway infection | |||||||
| Pseudomonas aeruginosa | 28.6 (75) | 39.9 (140) | 82.0 (553) | 65.3 (47) | 86.4 (108) | 32.4 (12) | 68.6 (35) |
| Burkholderia cepacia | 0 | 0 | 0 | 0 | 5.6 (7) | 100 (37) | 0 |
| MSSA | 89.7 (235) | 38.5 (135) | 34.7 (234) | 51.4 (37) | 28.0 (35) | 45.9 (17) | 45.1 (23) |
| MRSA | 2.3 (6) | 8.0 (28) | 23.4 (158) | 15.3 (11) | 19.2 (24) | 18.9 (7) | 25.5 (13) |
| Nontuberculous mycobacteria | 0 | 0 | 0 | 47.2 (34) | 0.8 (1) | 0 | 2.0 (1) |
| Comorbidities | |||||||
| Liver cirrhosis | 11.8 (31) | 2.6 (9) | 13.6 (92) | 11.1 (8) | 17.6 (22) | 16.2 (6) | 9.8 (5) |
| Diabetes mellitus | 0.4 (1) | 0.3 (1) | 36.1 (243) | 12.4 (9) | 40.8 (51) | 35.1 (13) | 41.2 (21) |
| Haemoptysis in 2005 | 0.4 (1) | 3.1 (11) | 15.3 (103) | 13.9 (10) | 22.4 (28) | 10.8 (4) | 15.7 (8) |
| Pneumothorax in 2005 | 0 | 0.3 (1) | 0 | 54.2 (39) | 10.4 (13) | 2.7 (1) | 2.0 (1) |
| Treated aspergillosis | 17.9 (47) | 15.7 (55) | 27.3 (184) | 22.2 (47) | 35.2 (44) | 27.0 (10) | 66.7 (34) |
| Treatment | |||||||
| Pancreatic enzyme | 92.0 (241) | 39.3 (138) | 95.8 (646) | 86.1 (62) | 97.6 (122) | 94.6 (35) | 86.3 (44) |
| Azithromycin | 20.6 (54) | 33.0 (116) | 67.5 (455) | 38.9 (28) | 78.4 (98) | 51.4 (19) | 58.8 (30) |
| Oral steroids | 0 | 0 | 0 | 0 | 2.4 (3) | 2.4 (1) | 100 (51) |
| Long-term oxygen therapy | 0 | 1.4 (5) | 1.6 (11) | 8.3 (6) | 81.6 (102) | 8.1 (3) | 23.5 (12) |
| Noninvasive ventilation | 0 | 0.3 (1) | 0 | 0 | 52.8 (66) | 0 | 7.8 (4) |
| Patients with i.v. antibiotics in 2005 | 26.7 (70) | 31.1 (109) | 69.6 (469) | 63.9 (46) | 93.6 (117) | 73.0 (27) | 72.5 (37) |
| Courses of i.v. antibiotics per patient in 2005 | 0 (0–1) | 0 (0–1) | 2 (0–3) | 1 (0–3) | 4 (3–5) | 1 (0.5–3) | 2 (0–4) |
| Patients hospitalised in 2005 | 14.9 (39) | 18.8 (66) | 32.8 (221) | 43.1 (31) | 76.8 (99) | 37.8 (14) | 51.0 (26) |
| Hospitalisations per patient in 2005 | 0 (0–0) | 0 (0–0) | 0 (0–1) | 0 (0–1) | 2 (1–4) | 0 (0–1) | 1 (0–2) |
| Thoracic surgery in 2005 | 1.1 (3) | 0.6 (2) | 1.3 (9) | 19.4 (14) | 1.6 (2) | 2.7 (1) | 0 |
Data are presented as n, % (n) or median (interquartile range). CFTR: CF transmembrane conductance regulator; FEV1: forced expiratory volume in 1 s; BMI: body mass index; MSSA: methicillin-susceptible Staphylococcus aureus; MRSA: methicillin-resistant S. aureus. #: missing n=70 (cluster 1 missing n=12; cluster 2 missing n=18; cluster 3 missing n=26; cluster 4 missing n=5; cluster 5 missing n=7; cluster 6 missing n=1; cluster 7 missing n=1); ¶: see supplementary table S2 for details on classification of CFTR mutations.