At diagnosis | |
Age years | 32 (27–44) |
Female | 13 (50) |
Current smokers | 26 (100) |
Pack-years | 16 (7–26) |
Dyspnoea | 16 (61) |
NYHA II/III/IV | 9/5/2 |
Pneumothorax | 8 (31) |
Lung function | |
TLC % pred | 96.8±12.0 |
FVC % pred | 86.7±20.1 |
RV % pred | 123.9±43.3 |
RV/TLC % pred | 125±38.8 |
FEV1 % pred | 83.6±20.7 |
FEV1/FVC % | 80.3±10.7 |
DLCO % pred# | 61.2±14.7 |
During follow-up | |
Follow-up duration months | 41 (26–84) |
Outcome status¶ | |
Persistent smoking | 14 (54) |
Improvement | 5 (35.7) |
Stability | 2 (14.3) |
Deterioration | 7 (50) |
Smoking cessation | 12 (46) |
Improvement | 8 (66.7) |
Stability | 2 (16.7) |
Deterioration | 2 (16.7) |