Clinical characteristics of the 26 patients with pulmonary Langerhans cell histiocytosis whose surgical lung biopsies were analysed
| 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) |
Data are presented as median (interquartile range), n (%) or mean±sd. PLCH: pulmonary Langerhans cell histiocytosis; NYHA: New York Heart Association; TLC: total lung capacity; FVC: forced vital capacity; RV: residual volume; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide. #: n=19; ¶: outcome status was assessed at the last time of follow-up based on the variations of dyspnoea (NYHA stage) and lung function. A variation ≥10% of FVC or FEV1 or ≥15% of DLCO was considered significant.