Table 2 Pulmonary Function Testing (PFTs)
PFTs at diagnosis (N = 40) | Last PFTs (N = 25) | Total mean change from first to last PFT, % predicted (N = 25) | Mean % change per year | P-value¶ | |
|---|---|---|---|---|---|
Obstruction, N (%) | 34 (85.0) | 21 (84.0) | |||
TLC% (median (IQR)) | 106 (88.0–115.0) | 112 (105.0–123.0) | 4.8 | 1.5 (N = 15) | 0.46 |
RV/TLC% (median (IQR)) | 158 (144.0–181.5) | 152 (146.3–166.8) | − 6.6 | − 1.7 (N = 14) | 0.07 |
FVC% (median (IQR)) | 66 (53.0–75.0) | 61 (54.5–74.5) | − 1.9 | − 0.5 (N = 25) | 0.42 |
FEV1% (median (IQR)) | 40 (31.0–52.5) | 41 (29.5–48.0) | −1.5 | −0.4 (N = 25) | 0.43 |
DLCO% (median (IQR)) | 69 (57.5–78.0) | 64 (57.0–76.8) | −2.3 | −0.6 (N = 18) | 0.34 |
Duration between first and last available PFT, months (median (IQR) and mean (SD)) | 33(14.6–51.7), 42 (40) |