TABLE 1

Clinical characteristics of the 26 patients with pulmonary Langerhans cell histiocytosis whose surgical lung biopsies were analysed

At diagnosis
 Age years32 (27–44)
 Female13 (50)
 Current smokers26 (100)
  Pack-years16 (7–26)
 Dyspnoea16 (61)
  NYHA II/III/IV9/5/2
 Pneumothorax8 (31)
 Lung function
  TLC % pred96.8±12.0
  FVC % pred86.7±20.1
  RV % pred123.9±43.3
  RV/TLC % pred125±38.8
  FEV1 % pred83.6±20.7
  FEV1/FVC %80.3±10.7
  DLCO % pred#61.2±14.7
During follow-up
 Follow-up duration months41 (26–84)
Outcome status
 Persistent smoking14 (54)
  Improvement5 (35.7)
  Stability2 (14.3)
  Deterioration7 (50)
 Smoking cessation12 (46)
  Improvement8 (66.7)
  Stability2 (16.7)
  Deterioration2 (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.