Figures
- Fig. 1—
Imaging of a typical case of combined pulmonary fibrosis and emphysema. a) Chest radiograph showing bilateral infiltrative opacities of the lower lobes and hyperlucent upper zones. b) Chest computed tomography (CT) of the upper zones of the lungs showing predominant centrilobular and paraseptal emphysema. c) Chest CT of the lower zones of the lungs showing reticular opacities, honeycombing, and traction bronchiectasis. Lung biopsy performed in this patient showed centrilobular emphysema of the upper lobes and usual interstitial pneumonia of the lower lobes.
- Fig. 2—
a) Survival of patients with combined emphysema and fibrosis (Kaplan-Meier analysis); 5-yr survival was 55%. b) Kaplan-Meier analysis of survival for subjects with combined emphysema and fibrosis stratified on the basis of pulmonary arterial hypertension (PAH) at diagnosis (––: no PAH, systolic arterial pulmonary pressure <45 mmHg, 5-yr survival 75%; ----: PAH, systolic arterial pulmonary pressure ≥45 mmHg, 5-yr survival 25%); p = 0.03.
Tables
- Table. 1—
Characteristics and clinical manifestations at diagnosis in 61 patients with combined pulmonary fibrosis and emphysema
Patient characteristics Sex M/F n 60/1 Age yrs 65.2±10.2 (36–84) Body mass index kg·m−2 26±3 (19–32) Current/ex-/never-smokers 19/42/0 Pack-yrs smoking 46±27 (5–120) Current smokers 57±27 (8–120) Ex-smokers 41±25 (5–110) Asthenia 14 (23) NYHA grade of dyspnoea Grade 1 10 (16) Grade 2 23 (38) Grade 3 23 (38) Grade 4 5 (8) Cough 29 (48) Sputum production 22 (36) Chest pain 10 (17) Finger clubbing 26 (43) Basal crackles 53 (87) Wheezes 8 (13) Data are presented as n (%) or mean±sd (range). M: male; F: female; NYHA: New York Heart Association.
- Table. 2—
Pulmonary function tests in patients with combined pulmonary fibrosis and emphysema
Test Patients tested n FVC % pred 61 90±18 (47–125) FEV1 % pred 61 80±21 (33–123) Post-bronchodilator improvement in FEV1 L 36 0.06±0.13 (−0.35–0.3) FEV1/FVC % 61 69±13 (30–94) FEF25–75% % pred 57 51±26 (15–118) TLC % pred 56 88±17 (44–132) RV % pred 56 90±32 (35–188) TL,CO % pred 57 37±16 (10–80) KCO % pred 57 46±19 (8–84) Pa,O2 at rest (supine position) kPa 61 8.4±1.9 (4.6–13.3) Pa,CO2 at rest (supine position) kPa 61 4.9±0.7 (3.0–7.3) Alveolar–arterial Pa,O2 difference (room air) kPa 61 5.5±2.1 (0.1–11.7) Pa,O2 at exercise–Pa,O2 at rest (supine position) kPa 22 −1.5±1.6 (−4.4–1.7) 6-min walking distance m 23 336±139 (50–548) Decrease in SP,O2 during 6-min walking test % 23 −8.9±5.7 (−20–0) Data are presented as mean±sd (range), unless otherwise stated. FVC: forced vital capacity; FEV1: forced expiratory volume in one second; FEF25–75%: mean forced expiratory flow between 25% and 75% of FVC; TLC: total lung capacity; RV: residual volume; TL,CO: transfer factor for carbon monoxide; KCO: transfer coefficient of the lung; Pa,O2: partial pressure of oxygen in arterial blood; Pa,CO2: partial pressure of carbon dioxide in arterial blood; SP,O2: arterial oxygen saturation measured by pulse oximetry.
- Table. 3—
Computed tomography (CT) findings
CT finding Fibrotic changes Honeycombing 58 (95) Reticular opacities 53 (87) Traction bronchiectasis 42 (69) Ground-glass opacities 40 (66) Architectural or bronchial distortion 24 (39) Emphysema Centrilobular emphysema 59 (97) Paraseptal emphysema 57 (93) Bullae 33 (54) Data are presented as n (%).
