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Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study

Marco Mura, Maria A. Porretta, Elena Bargagli, Gianluigi Sergiacomi, Maurizio Zompatori, Nicola Sverzellati, Amedeo Taglieri, Fabrizio Mezzasalma, Paola Rottoli, Cesare Saltini, Paola Rogliani
European Respiratory Journal 2012 40: 101-109; DOI: 10.1183/09031936.00106011
Marco Mura
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Maria A. Porretta
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Elena Bargagli
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Gianluigi Sergiacomi
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Maurizio Zompatori
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Nicola Sverzellati
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Amedeo Taglieri
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Fabrizio Mezzasalma
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Paola Rottoli
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Cesare Saltini
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Paola Rogliani
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  • For correspondence: paola.rogliani@uniroma2.it
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  • Figure 1–
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    Figure 1–

    Design of the study. IPF: idiopathic pulmonary fibrosis; VATS: video-assisted thoracoscopy; UIP: usual interstitial pneumonia; ATS: American Thoracic Society; ERS: European Respiratory Society.

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    Figure 2–

    a) Prospective cohort: 3-yr survival form the time of diagnosis. b) Prospective cohort: 3-yr incidence of acute exacerbations.

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    Figure 3–

    Prospective cohort, receiver operating characteristics curve of different variables at the time of diagnosis versus 3-yr survival (see table S3 for more details). a) Body mass index; b) Medical Research Council dyspnoea score; c) 6-min walking distance (6MWD; m); d) 6MWD (% predicted); e) alveolar–arterial oxygen tension difference; f) forced vital capacity; g) diffusing capacity of the lung for carbon monoxide; h) composite physiologic index; i) high-resolution computed tomography fibrosis score; and j) bronchoalveolar lavage total cell count.

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    Figure 4–

    a) Prospective cohort, Kaplan–Meier survival analysis grouped by Risk stratificatiOn ScorE (ROSE). Low risk: Medical Research Council dyspnoea score (MRCDS) ≤3, 6-min walking distance (6MWD) >72% predicted (% pred) and composite physiologic index (CPI) <41; intermediate risk: MRCDS >3, 6MWD <72% pred or CPI >41; and high risk: MRCDS >3, 6MWD <72% pred and CPI >41. p<0.0001. b) Prospective cohort, receiver operating characteristics curve of ROSE at the time of diagnosis versus 3-yr survival. Refer to table S3 for more details. c) Prospective cohort, intermediate risk group, Kaplan–Meier survival analysis grouped by 6-month changes of ROSE. p<0.0001. d) Prospective cohort, intermediate risk group, receiver operating characteristics curve of 6-month change of ROSE versus 3-yr survival; area under the curve 0.77, standard error 0.055, sensitivity 94% (95% CI 73–100%), specificity 41% (95% CI 25–58%). p-value <0.0001.

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  • Table 1– Demographic, clinical, functional and radiographic characteristics of the two cohorts# at the time of diagnosis
    VariableProspective cohortRetrospective cohortp-value
    Subjects n7068
    M/F (% male)57/13 (81)50/18 (74)ns
    Age yrs67±862±90.0011
    BMI m·kg−228±427±5ns
    Time to diagnosis months23±20NANA
    Biopsy-based diagnosis yes/no (%)23/47 (33)22/46 (32)ns
    Smokers/never-smokers (%)44/26 (63)34/34 (50)ns
    Smoking pack-yrs33±1824±170.028
    Concomitant emphysema yes/no (%)24/46 (34)13/55 (19)0.044
    MRC dyspnoea score2.5±1.1NANA
    6MWD¶ m372±146350±116ns
    6MWD¶ % pred79±2968±240.049
    PA–a,O2 mmHg32±1426±10ns
    FVC % pred75±2275±21ns
    DL,CO+ % pred46±1955±170.013
    CPI48±1542±120.038
    HRCT disease extent§ % of total lung volume36±12NANA
    BAL total cell countƒ cells·mL−1596130±502073NANA
    • Data are presented as mean±sd, unless otherwise stated. M: male; F: female; BMI: body mass index; MRC: Medical Research Council; 6MWD: 6-min walking distance; % pred: % predicted; PA–a,O2: alveolar–arterial oxygen tension difference; FVC: forced vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; CPI: composite physiologic index; HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage; ns: not significant; NA: not available. #: a prospective cohort at the Pulmonary Unit, University of Rome “Tor Vergata”, Rome, Italy and a retrospective cohort at the pulmonary unit, University of Siena, Siena, Italy; ¶: n=64; +: n=65; §: mean of the scores from each of the three core radiologists; ƒ: n=28. p-value: Chi-squared, unpaired t-test or Mann–Whitney U-test, where required.

  • Table 2– Prospective cohort: demographic, clinical, functional and radiographic characteristics at the time of diagnosis of 3-yr survivors and nonsurvivors
    VariableSurvivorsNonsurvivorsp-value
    Subjects n3733
    Age yrs66±467±9ns
    BMI m·kg−229±427±30.0384
    Time to diagnosis months20±1927±22ns
    Smoking pack-yrs32±1634±22ns
    Concomitant emphysema yes/no (%)9/28 (24)15/18 (45)0.0267
    MRC dyspnoea score2.1±0.83.3±1.1<0.0001
    6MWD m412±128319±1530.0184
    6MWD % pred89±2766±270.0018
    Desaturation at 6MWT# yes/no (%)19/16 (54)25/4 (86)0.0061
    PA–a,O2 mmHg28±1336±170.0392
    FVC % pred81±2168±230.0027
    DL,CO % pred53±1737±170.0005
    CPI43±1555±130.0006
    HRCT disease extent % of total lung volume33±1141±110.0098
    BAL total cell count cells·mL−1417972±319004952444±6235050.0037
    • Data are presented as mean±sd, unless otherwise stated. BMI: body mass index; MRC: Medical Research Council; 6MWD: 6-min walking distance; % pred: % predicted; 6MWT: 6-min walking test; PA–a,O2: alveolar–arterial oxygen tension difference; FVC: forced vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; CPI: composite physiologic index; HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage; ns: not significant. p-value: Chi-squared, unpaired t-test or Mann–Whitney U-test, where required. #: arterial oxygen saturation ≤88%.

  • Table 3– Prospective cohort: 3-yr survival, univariate analysis of variables at the time of diagnosis
    VariableHazard ratio (95% CI)p-value
    Age yrs1.02 (0.98–1.07)ns
    Smoking pack-yrs0.99 (0.97–1.02)ns
    Time to diagnosis months1.01 (0.99–1.03)ns
    BMI m·kg−20.89 (0.80–0.98)0.0155
    MRC dyspnoea score2.52 (1.76–3.62)<0.0001
    6MWD m1.00 (1.00–1.00)ns
    6MWD % pred0.98 (0.96–0.99)0.0007
    Desaturation at 6MWT#0.54 (0.32–0.84)0.0052
    PA-a,O2 mmHg1.03 (1.01–1.05)0.0063
    FVC % pred0.97 (0.95–0.99)0.0039
    DL,CO % pred0.94 (0.92–0.97)<0.0001
    CPI1.06 (1.03–1.10)<0.0001
    HRCT fibrosis score % of total lung volume1.03 (1.00–1.07)0.0497
    BAL total cell count cells·mL−11.00 (1.00–1.00)0.0009
    Concomitant emphysema1.82 (0.90–3.66)0.096
    • BMI: body mass index; MRC: Medical Research Council; 6MWD: 6-min walking distance; % pred: % predicted; 6MWT: 6-min walking test; PA–a,O2: alveolar–arterial oxygen tension difference; FVC: forced vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; CPI: composite physiologic index; HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage; ns: not significant. #: no desaturation (arterial oxygen saturation ≤88%) during the 6MWT.

  • Table 4– Prospective and retrospective cohorts: 3-yr survival Cox proportional hazard analysis of variables at the time of diagnosis
    VariableHazard ratio (95% CI)p-value
    Prospective cohort#
     MRC dyspnoea score >36.77 (2.37–19.41)0.0005
     6MWD <72% pred3.27 (1.25–8.82)0.0162
     CPI >415.36 (1.50–34.15)0.0071
    Retrospective cohort¶
     6MWD <72% pred5.43 (1.35–36.17)0.016
     CPI >414.20 (1.05–27.93)0.042
    • MRC: Medical Research Council; 6MWD: 6-min walking distance; % pred: % predicted; CPI: composite physiologic index. #: p-value of the model <0.0001; ¶: p-value of the model 0.0053.

  • Table 5– Prospective cohort: acute exacerbations, univariate analysis of variables at the time of diagnosis
    VariableHazard ratio (95% CI)p-value
    Age at diagnosis yrs1.02 (0.96–1.10)ns
    Smoking pack-yrs0.99 (0.95–1.03)ns
    Time to diagnosis months1.01 (0.98–1.04)ns
    BMI m·kg−20.92 (0.79–1.07)ns
    MRC dyspnoea score1.96 (1.18–3.30)0.0095
    6MWD m1.00 (1.00–1.00)ns
    6MWD % pred0.98 (0.96–1.00)ns
    Desaturation at 6MWT#0.43(0.10–0.97)0.040
    PA–a,O2 mmHg1.03(1.00–1.07)0.053
    FVC % pred0.98 (0.95–1.01)ns
    DL,CO % pred0.94 (0.90–0.98)0.0007
    CPI1.05 (1.01–1.10)0.022
    HRCT fibrosis score % of total lung volume1.05 (1.00–1.10)0.063
    BAL total cell count cells·mL−11.00 (1.00–1.00)ns
    Concomitant emphysema3.37 (1.12–11.17)0.030
    • BMI: body mass index; MRC: Medical Research Council; 6MWD: 6-min walking distance; % pred: % predicted; 6MWT: 6-min walking test; PA–a,O2: alveolar–arterial oxygen tension difference; FVC: forced vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; CPI: composite physiologic index; HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage; ns: not significant. #: no desaturation (arterial oxygen saturation ≤88%) during the 6MWT.

  • Table 6– Prospective cohort: acute exacerbations, Cox proportional hazard analysis of variables at the time of diagnosis
    VariableHazard ratio (95% CI)p-value
    DL,CO % pred0.93 (0.89–0.97)0.0008
    Concomitant emphysema3.20 (1.06–10.67)0.040
    • DL,CO: diffusing capacity of the lung for carbon monoxide; % pred: % predicted. Model 1 (cut-off), p-value of the model 0.0004.

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Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study
Marco Mura, Maria A. Porretta, Elena Bargagli, Gianluigi Sergiacomi, Maurizio Zompatori, Nicola Sverzellati, Amedeo Taglieri, Fabrizio Mezzasalma, Paola Rottoli, Cesare Saltini, Paola Rogliani
European Respiratory Journal Jul 2012, 40 (1) 101-109; DOI: 10.1183/09031936.00106011

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Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study
Marco Mura, Maria A. Porretta, Elena Bargagli, Gianluigi Sergiacomi, Maurizio Zompatori, Nicola Sverzellati, Amedeo Taglieri, Fabrizio Mezzasalma, Paola Rottoli, Cesare Saltini, Paola Rogliani
European Respiratory Journal Jul 2012, 40 (1) 101-109; DOI: 10.1183/09031936.00106011
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