Table 1.
Patient characteristics at first measurement of DLCO < 50% of predicted
| n = 106 | ||
|---|---|---|
| Age | 49 ± 13 years | |
| Male/Female | 72 (68)/34 (32) | |
| White/ Non-white/Unknown | 66 (62)/30 (28)/10 (9) | |
| Histologic confirmation | 104 (98) | |
| Smoking history | Never | 29 (27) |
| Former | 48 (45) | |
| Current | 23 (22) | |
| Unknown | 6 (6) | |
| Cardiac involvement | Probable | 6 (6) |
| Possible | 2 (2) | |
| ≥ 2 organs involved | 95 (90) | |
| Therapy | Corticosteroids | 59 (56) |
| Methotrexate | 37 (35) | |
| Azathioprine | 3 (3) | |
| Plaquenil | 4 (4) | |
| Anti-TNF treatment | 8 (8) | |
| None | 26 (25) | |
| Unknown | 4 (4) | |
| Duration of disease prior to baseline time point | 5 (13) years | |
| Scadding stage | 0 | 2 (2) |
| I | 4 (4) | |
| II | 19 (18) | |
| III | 12 (11) | |
| IV | 63 (59) | |
| Unknown | 6 (6) | |
| PH | Diagnosed with RHC | 9 (8) |
| PH suspected on echocardiogram | 3 (3) | |
| FVC % predicted | 70 ± 17 | |
| FEV1% predicted | 60 ± 18 | |
| DLCOc% predicted | 42 ± 7 | |
| CPI | 47 ± 9 | |
| Walsh poor prognosis | 98 (92) | |
| MUC5B promotor polymorphism | GG | 83 (78) |
| GT | 23 (22) | |
| TT | 0 | |
Data are shown as number (%) or mean ± SD, except for the duration of disease this is shown as median (IQR)
PH = pulmonary hypertension; FVC = forced vital capacity; FEV1 = forced expiratory volume in one second; DLCOc = diffusing capacity for carbon monoxide corrected for haemoglobin; RHC = right heart catheterization; CPI = composite physiologic index; MUC5B = Mucin 5B rs35705950