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Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity

V. Cottin, H. Nunes, P-Y. Brillet, P. Delaval, G. Devouassoux, I. Tillie-Leblond, D. Israel-Biet, I. Court-Fortune, D. Valeyre, J-F. Cordier
European Respiratory Journal 2005 26: 586-593; DOI: 10.1183/09031936.05.00021005
V. Cottin
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H. Nunes
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P-Y. Brillet
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P. Delaval
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G. Devouassoux
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I. Tillie-Leblond
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D. Israel-Biet
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I. Court-Fortune
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D. Valeyre
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J-F. Cordier
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  • Fig. 1—
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    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—
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    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

  • Figures
  • Table. 1—

    Characteristics and clinical manifestations at diagnosis in 61 patients with combined pulmonary fibrosis and emphysema

    Patient characteristics
    Sex M/F n60/1
    Age yrs65.2±10.2 (36–84)
    Body mass index kg·m−226±3 (19–32)
    Current/ex-/never-smokers19/42/0
    Pack-yrs smoking46±27 (5–120)
    Current smokers57±27 (8–120)
    Ex-smokers41±25 (5–110)
    Asthenia14 (23)
    NYHA grade of dyspnoea
    Grade 110 (16)
    Grade 223 (38)
    Grade 323 (38)
    Grade 45 (8)
    Cough29 (48)
    Sputum production22 (36)
    Chest pain10 (17)
    Finger clubbing26 (43)
    Basal crackles53 (87)
    Wheezes8 (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

    TestPatients tested n
    FVC % pred6190±18 (47–125)
    FEV1 % pred6180±21 (33–123)
    Post-bronchodilator improvement in FEV1 L360.06±0.13 (−0.35–0.3)
    FEV1/FVC %6169±13 (30–94)
    FEF25–75% % pred5751±26 (15–118)
    TLC % pred5688±17 (44–132)
    RV % pred5690±32 (35–188)
    TL,CO % pred5737±16 (10–80)
    KCO % pred5746±19 (8–84)
    Pa,O2 at rest (supine position) kPa618.4±1.9 (4.6–13.3)
    Pa,CO2 at rest (supine position) kPa614.9±0.7 (3.0–7.3)
    Alveolar–arterial Pa,O2 difference (room air) kPa615.5±2.1 (0.1–11.7)
    Pa,O2 at exercise–Pa,O2 at rest (supine position) kPa22−1.5±1.6 (−4.4–1.7)
    6-min walking distance m23336±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
     Honeycombing58 (95)
     Reticular opacities53 (87)
     Traction bronchiectasis42 (69)
     Ground-glass opacities40 (66)
     Architectural or bronchial distortion24 (39)
    Emphysema
     Centrilobular emphysema59 (97)
     Paraseptal emphysema57 (93)
     Bullae33 (54)
    • Data are presented as n (%).

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Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity
V. Cottin, H. Nunes, P-Y. Brillet, P. Delaval, G. Devouassoux, I. Tillie-Leblond, D. Israel-Biet, I. Court-Fortune, D. Valeyre, J-F. Cordier
European Respiratory Journal Oct 2005, 26 (4) 586-593; DOI: 10.1183/09031936.05.00021005

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Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity
V. Cottin, H. Nunes, P-Y. Brillet, P. Delaval, G. Devouassoux, I. Tillie-Leblond, D. Israel-Biet, I. Court-Fortune, D. Valeyre, J-F. Cordier
European Respiratory Journal Oct 2005, 26 (4) 586-593; DOI: 10.1183/09031936.05.00021005
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