Demographics and clinical characteristics of patients 1–4 with IPF
| Characteristic . | Patient 1 . | Patient 2 . | Patient 3 . | Patient 4 . |
|---|---|---|---|---|
| Sex | M | M | M | M |
| Age at diagnosis (yr) | 32 | 57 | 72 | 52 |
| FVC (% predicted) | 89 | 78 | 102 | 62 |
| DLCOc (% predicted) | 40 | 65 | 75 | 47 |
| Smoking behavior | Former | Former | Former | Never |
| Pack years | 7 | 27 | 30 | NA |
| HRCT pattern | Probable UIP | Probable UIP | Probable UIP | Probable UIP |
| Inspiratory crackles | Yes | Yes | No | Yes |
| Digital clubbing | No | Yes | No | No |
| Additional STS | Liver disease | Peritoneal fibrosis | Liver disease; thrombocytopenia, RBC macrocytosis | No |
| T/Sobs-exp | −0.488 | −0.301 | −0.236 | −0.204 |
| T/S <10th percentile | Yes | Yes | Yes | Yes |
| Characteristic . | Patient 1 . | Patient 2 . | Patient 3 . | Patient 4 . |
|---|---|---|---|---|
| Sex | M | M | M | M |
| Age at diagnosis (yr) | 32 | 57 | 72 | 52 |
| FVC (% predicted) | 89 | 78 | 102 | 62 |
| DLCOc (% predicted) | 40 | 65 | 75 | 47 |
| Smoking behavior | Former | Former | Former | Never |
| Pack years | 7 | 27 | 30 | NA |
| HRCT pattern | Probable UIP | Probable UIP | Probable UIP | Probable UIP |
| Inspiratory crackles | Yes | Yes | No | Yes |
| Digital clubbing | No | Yes | No | No |
| Additional STS | Liver disease | Peritoneal fibrosis | Liver disease; thrombocytopenia, RBC macrocytosis | No |
| T/Sobs-exp | −0.488 | −0.301 | −0.236 | −0.204 |
| T/S <10th percentile | Yes | Yes | Yes | Yes |
DLCOc, diffusing capacity of the lungs for carbon monoxide corrected for hemoglobin concentration; FVC, forced vital capacity; STS, short telomere syndrome; T/Sobs-exp, telomere length adjusted for age calculated by the difference between observed T/S ratio and the age-adjusted normal value.