Table 1 Baseline characteristics for 409 patients with idiopathic pulmonary fibrosis
Total patients | 409 |
|---|---|
Male (%) | 313 (77) |
Median age at diagnosis (IQR) | 68.2 (62.0–74.5) |
First-degree family member with pulmonary fibrosis (%) | 116 (28) |
Other family member with pulmonary fibrosis (%) | 24 (6) |
Current smoker (%) | 33 (8) |
Former smoker (%) | 298 (73) |
Never smoker (%) | 78 (19) |
Significant exposure to asbestos, dusts, fumes, or radiation (%) | 79 (9) |
Gastro-oesophageal reflux (%) | 149 (36) |
Cardiovascular disease (%) | 188 (46) |
Initial symptoms | |
Cough | 284 (69) |
Dyspnoea | 343 (84) |
No respiratory complaints | 9 (2) |
Evaluation in context of screening of family members of patients with familial pulmonary fibrosis | 5 (1) |
Chest X-ray done prior to start of symptoms (%) | 96 (23) |
Showing potential interstitial lung abnormalities (%) | 56 (58) |
CT-imaging done prior to start of symptoms (%) | 44 (11) |
Showing interstitial lung abnormalities or pulmonary fibrosis (%) | 34 (77) |
Symptoms started directly after infectious episode (%) | 40 (10) |
Symptoms started directly after surgery (%) | 5 (1) |
Treatment with steroids prior to diagnosis (%) # | 103 (25) |
Treatment with other immunosuppressant prior to diagnosis (%) | 23 (6) |
Other ILD diagnosis prior to diagnosis of IPF (%) | 67 (16) |
Cardiology workup for symptoms prior to pulmonology visit (%) | 45 (11) |
Time from start of symptoms to first visit with pulmonologist, median months (IQR) | 5.0 (2.0–12.0) |
Time from first visit with pulmonologist to start of symptoms, median months (IQR) | 10.0 (4.0–33.0) |
Time from start of symptoms to diagnosis, median months (IQR) | 24.0 (9.0–48.0) |
Forced vital capacity at diagnosis, median percentage of predicted (IQR) | 78.1 (64.0–91.3) |
Diffusion capacity for carbon monoxide at diagnosis, median percentage of predicted (IQR) | 41.0 (32.0–51.0) |
Radiographic pattern at diagnosis | |
UIP pattern (%) | 238 (58) |
Probable UIP (%) | 101 (25) |
Inconsistent with UIP or indeterminate for UIP (%) | 70 (17) |
Histology pattern at diagnosis | 99 (24) |
UIP pattern (%) * | 70 (70) |
Probable UIP (%) * | 16 (16) |
Inconsistent with UIP or indeterminate for UIP (%) * | 13 (13) |
Consensus diagnosis of IPF (%) | 190 (47) |
Received treatment with antifibrotic therapy after diagnosis (%) | 331 (81) |