Table 2– Pulmonary function tests, serum angiotensin-converting enzyme (SACE), high-resolution computed tomography (HRCT) and bronchoalveolar lavage (BAL) findings at first evaluation with radiographic stage IV disease
Spirometry#
 FEV1 mL1921±631 (660–3520)
 FEV1 % pred63.9±20.7 (21–114)
 FEV1/FVC %73.4±14 (34–99)
 FVC mL2672±857 (740–5330)
 FVC % pred71.6±22.4 (23–137)
 TLC mL4232±1049 (1510–7460)
 TLC % pred73.8±18.6 (34–116)
DL,CO
DL,CO % pred56.2±17.8 (18–101)
KCO % pred81.5±20 (31–135)
Room-air blood gases+
Pa,O2 mmHg79.4±11.7 (42–111)
Pa,CO2 mmHg39.7±5.2 (30–79)
SACE level§
 ≤1 ULN52 (40.3)
 >1 and ≤2 ULN40 (31)
 >2 ULN37 (28.7)
HRCT pattern of fibrosisƒ,##
 Bronchial distortion85 (64.4)
 Linear27 (20.5)
 Honeycombing15 (11.4)
 Unclassified5 (3.8)
BAL
 Total cell count cells·mm−3442±62 (28–4000)
 Lymphocytes %29±19.9 (1–73.4)
 Lymphocytosis >15%29 (64.4)
 Neutrophils %16.9±23.8 (0–96)
 Eosinophils %1±1.4 (0–7)
  • Data are presented as mean±sd (range) or n (%). FEV1: forced expiratory volume in 1 s; % pred: % predicted; FVC: forced vital capacity; TLC: total lung capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; KCO: transfer coefficient of the lung for carbon monoxide (DL,CO/alveolar volume); Pa,O2: arterial oxygen tension; Pa,CO2: arterial carbon dioxide tension; ULN: upper limit of normal. #: n=133; : n=118; +: n=126; §: n=129; ƒ: HRCT pattern of pulmonary fibrosis according to Abehsera et al. [24]; ##: n=132.