Table 1.
Clinical characteristics of 131 IPF patients and 43 iPPFE patients.
| IPF (n = 131) UIP/IPF 50 (38.2%), cIPF 81(61.8%) | iPPFE (n = 43) | |
|---|---|---|
| Age, yr | 69.0 [64.0–75.0] | 69.0 [64.0–74.0] |
| Sex, male/female | 117 (89.3%)/14 (10.7%) | 27 (62.8%)/16 (37.2%) |
| Observation period, mo | 53.3 [31.6–86.1] | 31.3 [18.2–47.2] |
| Never or former/current smoker | 20 (15.3%), 111 (84.7%) | 29 (67.4%), 14 (32.6%) |
| Smoking pack-year | 35.0 [20.0–60.0] | 0 [0–12.5] |
| acute exacerbation, yes | 39 (29.8%) | 8 (18.6%) |
| Height, cm | 162.1 [157.0–166.0] | 159.0 [152.0–165.0] |
| Weight, kg | 60.0 [52.1–67.0] | 42.3 [35.8–48.0] |
| BMI, kg/m2 | 23.1 [21.3–24.7] | 17.2 [14.7–18.5] |
| ESMCSA, cm2 | 32.8 [27.1–37.7] | 23.4 [17.8–30.6] |
| ESMMA, HU | 42.0 [35.7–45.7] | 43.5 [38.2–48.7] |
| Pulmonary Function Test | ||
| FVC, %-pred | 80.5 [66.4–92.9] (n = 120) | 54.4 [45.8–67.5] (n = 37) |
| FEV1/FVC, % | 83.5 [79.4–88.0] (n = 120) | 96.3 [91.4–100] (n = 37) |
| DLCO, % | 68.6 [55.4–97.1] (n = 52) | 68.7 [47.9–91.9] (n = 24) |
| Laboratory | ||
| PaO2, Torr | 80.0 [73.0–89.1] (n = 111) | 79.0 [71.5–84.9] (n = 38) |
| PaCO2, Torr | 41.9 [39.0–44.0] (n = 111) | 46.7 [41.3–49.0] (n = 38) |
| Alb, g/dl | 4.0 [3.8–4.3] (n = 124) | 4.0 [3.5–4.1] (n = 40) |
| KL-6, U/ml | 868.5 [547.3–1240.5] (n = 114) | 503.0 [365.0–638.8] (n = 42) |
| SP-D ng/ml | 203.0 [133–316] (n = 111) | 186.0 [134.3–269.5] (n = 40) |
BMI; body mass index, ESMCSA; cross-sectional area of elector spine muscles, ESMMA; muscle attenuation of elector spine muscles, FVC; forced vital capacity, FEV1.0; forced expiratory volume in 1.0 second, DLCO; diffuse capacity of the lung for carbon monoxide, KL-6; Krebs von den Lunge-6, SP-D; surfactant protein-D.