Table 1 Characteristics of PLCH patients included in the study
Characteristics | Diffuse cystic pattern n = 24 | Nodulo-cystic pattern n = 26 |
|---|---|---|
Age, years, median, [IQR] | 40 [31–46.75] | 45 [34–47] |
Female sex, n (%) | 13 (54%) | 17 (65%) |
Histological diagnosis | 13 (54%) | 3 (12%) |
Isolated PLCH, n (%) | 15 (62.5%) | 23 (88%) |
MS LCH, n (%) | 9 (37.5%) | 3 (12%) |
Time from PLCH diagnosis, years, median, [IQR] | 1 [0–10] | 0 [0–5] |
Smoker, n (%) | 15 (62.5%) | 24 (92%) |
Ex-smoker | 9 (37.5%) | 2 (8%) |
Dyspnoea | 18 (75%) | 16 (62%) |
NYHA II/III/IV | 9/8/1 | 14/2 |
TLC, % of predicted | 104 [92.75–123.75] | 105 [95.25–123] |
FVC, % of predicted | 78 [69.25–87.25] | 100 [84.25–114] |
RV/TLC, % of predicted | 148.5 [118.5–166.75] | 111.5 [103–132.5] |
FEV1, % of predicted | 58.5 [39.5–69.5] | 86.5 [76–100] |
FEV1/FVC % | 66 [48.25–72] | 77 [69.25–80.75] |
DLCO, % of predicted, n = 23 | 37 [28–56] | 52 [41–63] |
Restriction* (%) | 0 | 0 |
Obstruction (%) | 17 (71%) | 7 (27%) |
Air trapping (%) | 17 (71%) | 10 (38%) |
CT cyst score, median, [IQR] | 20 [16.5–22] | N/A |
Alpha-1 antitrypsin level (g/L) (mean, SD) | 1.39 (± 0.37) | 1.41 (± 0.21) |
PiM allele frequency | 94% | 100% |
PiS allele frequency | 4% | 0% |
PiZ allele frequency | 2% | 0% |