Table 2.
Evaluations performed before and after referral to enrolling site, by time to diagnosis
| N | Overall analysis cohort | Time from symptom onset to diagnosis | Time from first imaging evidence of pulmonary fibrosis to diagnosis | ||
| ≤1 year | >1 year | ≤1 year | >1 year | ||
| 498 | 174 | 173 | 356 | 98 | |
| Evaluations prior to referral* | |||||
| Surgical lung biopsy | 66 (13.9%) | 21 (12.4%) | 26 (15.8%) | 39 (11.4%) | 15 (16.5%) |
| Where time of evaluation known: | |||||
| Within 12 months prior to referral | 46 (9.7%) | 17 (10.1%) | 15 (9.1%) | 31 (9.1%) | 9 (9.9%) |
| HRCT | 339 (71.1%) | 115 (67.6%) | 122 (73.5%) | 238 (69.4%) | 75 (82.4%) |
| Where time of evaluation known: | |||||
| Within 12 months prior to referral | 261 (56.4%) | 94 (56.0%) | 86 (54.1%) | 210 (62.1%) | 36 (40.9%) |
| Seen by a pulmonary specialist | 224 (45.1%) | 79 (45.4%) | 97 (56.4%) | 142 (39.9%) | 57 (58.8%) |
| Where time of evaluation known: | |||||
| Within 12 months prior to referral | 164 (34.2%) | 73 (42.4%) | 60 (35.9%) | 119 (34.5%) | 27 (29.3%) |
| Cardiac catheterisation | 87 (18.3%) | 28 (16.5%) | 34 (20.9%) | 61 (17.8%) | 16 (17.8%) |
| Where time of evaluation known: | |||||
| Within 12 months prior to referral | 25 (5.3%) | 6 (3.6%) | 7 (4.3%) | 21 (6.2%) | 3 (3.4%) |
| Evaluations in 12 months prior to referral | |||||
| Echocardiogram | 165 (34.8%) | 56 (32.9%) | 57 (34.8%) | 117 (34.3%) | 33 (36.7%) |
| Cardiac stress test | 105 (22.0%) | 36 (21.2%) | 38 (22.9%) | 73 (21.3%) | 20 (22.0%) |
| Serologies for connective tissue disease | 114 (23.9%) | 37 (21.8%) | 47 (28.5%) | 87 (25.4%) | 19 (20.9%) |
| Environmental or occupational exposures | 115 (24.2%) | 35 (20.8%) | 42 (25.5%) | 83 (24.3%) | 23 (25.6%) |
| Exposure to drugs with known pulmonary toxicity | 89 (18.8%) | 25 (14.8%) | 33 (20.0%) | 62 (18.2%) | 19 (21.1%) |
| Bronchoscopy | 35 (7.4%) | 17 (10.0%) | 15 (9.0%) | 23 (6.7%) | 7 (7.7%) |
| Referral for lung transplant evaluation | 9 (1.9%) | 3 (1.8%) | 5 (3.0%) | 8 (2.3%) | 0 |
| Evaluations at the enrolling centre | |||||
| Diagnostic criteria22 | |||||
| Definite IPF | 321 (64.5%) | 111 (63.8%) | 109 (63.0%) | 233 (65.4%) | 66 (67.3%) |
| Probable IPF | 137 (27.5%) | 48 (27.6%) | 52 (30.1%) | 95 (26.7%) | 25 (25.5%) |
| Possible IPF | 40 (8.0%) | 15 (8.6%) | 12 (6.9%) | 28 (7.9%) | 7 (7.1%) |
| HRCT | 352 (71.3%) | 119 (68.8%) | 133 (77.3%) | 237 (67.1%) | 78 (80.4%) |
| Clinically significant emphysema on HRCT | 62 (12.5%) | 17 (9.8%) | 23 (13.3%) | 41 (11.6%) | 17 (17.5%) |
| Pulmonary function tests | 482 (96.8%) | 170 (97.7%) | 168 (97.1%) | 343 (96.3%) | 96 (98.0%) |
| Serologies for connective tissue disease | 291 (61.3%) | 100 (59.2%) | 112 (68.3%) | 196 (57.3%) | 64 (70.3%) |
| Echocardiogram | 129 (27.0%) | 37 (21.8%) | 57 (34.5%) | 77 (22.4%) | 37 (40.7%) |
| Cardiac catheterisation | 30 (6.3%) | 10 (5.9%) | 10 (6.1%) | 21 (6.1%) | 7 (7.7%) |
| Multidisciplinary discussion | 218 (43.8%) | 75 (43.1%) | 84 (48.6%) | 142 (39.9%) | 52 (53.1%) |
*Time period of evaluation (prior) was unknown for 3% of surgical lung biopsies, 4% of HCRT, 8% of pulmonary specialist referrals and 5% of cardiac catheterisations.
HRCT, high-resolution CT; IPF, idiopathic pulmonary fibrosis.