TABLE 6.
Comparison of probable and definite familial interstitial pneumonia
| Probable | Definite | |
|---|---|---|
| Demographic and Clinical Characteristics | (n = 231) | (n = 78) |
| Age, yr, mean ± SD | 69.6 ± 11.2 | 64.5 ± 9.4* |
| Range (median) | 30.3–95.4 (71.2) | 35.8–89.4 (63.4) |
| Male sex, no. (%) | 124 (53.7) | 48 (61.5) |
| Age at diagnosis, yr, mean ± SD | 68.0 ± 11.1 | 62.6 ± 9.7* |
| Range (median) | 29.8–94.4 (69.3) | 30.9–89.1 (62.4) |
| Mortality: deaths, no. (%) | 102 (44.2) | 48 (61.5)† |
| Age at death, yr, mean ± SD | 68.8 ± 10.8 | 64.7 ± 9.3‡ |
| Range (median) | 35.8–87.9 (70.4) | 47.3–82.3 (64.2) |
| Time to death from age at diagnosis, yr, mean ± SD | 1.7 ± 2.1 | 1.0 ± 1.3‡ |
| Cigarette smoking history | n = 188 | n = 44 |
| Current smoker, no. (%) | 14 (7.4) | 4 (9.1) |
| Former smoker, no. (%) | 113 (60.1) | 25 (56.8) |
| Never smoker, no. (%) | 61 (32.4) | 15 (34.1) |
| Pack-yr, mean ± SD | 16.6 ± 16.2 | 15.4 ± 15.4 |
| Range (median) | 0–62 (15) | 0–42 (16) |
| Mean no. of cigarettes/d | 12.0 ± 12.0 | 13.9 ± 13.0 |
| Range (median) | 0–60 (10) | 0–40 (13) |
| Dyspnea | ||
| Dyspnea score, mean ± SD | 2.7 ± 1.9 | 2.8 ± 2.1 |
| Range (median) | 0–5 (3) | 0–5 (4) |
| Dyspnea class 0 (none), no. (%) | 40 (21.9) | 11 (28.2) |
| Dyspnea class 1–2 (mild), no. (%) | 44 (24.0) | 6 (15.4) |
| Dyspnea class 3–4 (moderate), no. (%) | 55 (30.1) | 7 (17.9) |
| Dyspnea class 5 (severe), no. (%) | 44 (24.0) | 15 (38.5) |
| Vital capacity, %pred: mean ± SD (no.) | 69.4 ± 18.7 (98) | 63.4 ± 22.5 (32) |
| Range (median) | 28–129 (69.5) | 19–97 (63.0) |
| DlCO, %pred: mean ± SD (no.) | 49.8 ± 20.5 (182) | 46.2 ± 20.0 (58) |
| Range (median) | 3–132 (50.0) | 14–96 (43.5) |
Definition of abbreviation: DlCO = carbon monoxide diffusing capacity.
*
p = 0.006.
†
p < 0.0001.
‡
p = 0.02.