Table 1—

Characteristics, clinical manifestations, and pulmonary function tests at diagnosis in patients with combined pulmonary fibrosis and emphysema syndrome

Sex male/female n38/2
Age yrs68.2±8.9 (47.7–82.2)
Current/ex/never-smokers n2/37/1
Smoking history pack-yrs46±23 (17–120)
NYHA class I0
NYHA class II6 (15)
NYHA class III22 (55)
NYHA class IV12 (30)
6MWD m244±126 (78–689)
6MWT Sp,O2 at end of test77±10 (62–96)
6MWT Sp,O2 decrease during test-15±8 (-30– -1)
FVC % pred86±18 (46–116)
FEV1 % pred78±19 (38–114)
FEV1/FVC %75±18 (29–107)
TLC % pred84±23 (47–139)
RV % pred87±47 (41–219)
DL,CO % pred24±14 (3–52)
KCO % pred28±16 (4–68)
Pa,O2 at rest kPa7.5±1.6 (5.2–11.7)
Pa,O2 at rest mmHg56.2±12.0 (39–84)
Pa,CO2 at rest kPa4.7±0.6 (3.3–5.9)
Pa,CO2 at rest mmHg35.2±4.5 (25–44)
  • Data are presented as mean±sd (range) or n (%), unless otherwise stated. NYHA: New York Heart Association; 6MWD: 6-min walk distance; 6MWT: 6-min walk test; Sp,O2: peripheral oxygen saturation; FVC: forced vital capacity; % pred: % predicted; FEV1: forced expiratory volume in 1 s; TLC: total lung capacity; RV: residual volume; DL,CO: single-breath diffusing capacity of the lung for carbon monoxide; KCO: single breath transfer factor of the lung for carbon monoxide; Pa,O2: arterial oxygen tension; Pa,CO2: arterial carbon dioxide tension.