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Staging of idiopathic pulmonary fibrosis: past, present and future

Martin Kolb, Harold R. Collard
European Respiratory Review 2014 23: 220-224; DOI: 10.1183/09059180.00002114
Martin Kolb
1Dept of Medicine, Firestone Institute for Respiratory Health, St. Joseph’s Healthcare, McMaster University, Hamilton, ON, Canada. 2Dept of Medicine, Division of Pulmonary, Critical Care Medicine, Sleep and Allergy, University of California, San Francisco, CA, USA
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Harold R. Collard
1Dept of Medicine, Firestone Institute for Respiratory Health, St. Joseph’s Healthcare, McMaster University, Hamilton, ON, Canada. 2Dept of Medicine, Division of Pulmonary, Critical Care Medicine, Sleep and Allergy, University of California, San Francisco, CA, USA
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  • For correspondence: hal.collard@ucsf.edu
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  • Table 1. Mortality risk scoring system for patients with idiopathic pulmonary fibrosis
    Risk factorsScore
    Age years
     ≥708
     60–694
     <600
    Recent respiratory hospitalisation
     Yes14
     No0
    Baseline FVC % predicted
     ≤5018
     51–6513
     66–798
     ≥800
    24-week change in FVC % predicted
     ≤ -1021
     -5 to -9.910
     > -4.90
    • FVC: forced vital capacity. Sum the individual scores corresponding to the level of each risk factor for a given patient, e.g. the total score for a patient aged 70 years, with no history of respiratory hospitalisation, FVC 51–65% predicted, and a 24-week change in FVC of -5 to -9.9% predicted is 8+0+13+10=31. Reproduced with modification from [21] with permission from the publisher.

  • Table 2. Expected 1-year probability of death in patients with idiopathic pulmonary fibrosis
    Total risk scoreExpected 1-year probability of death %
    0–4<2
    8–142–5
    16–215–10
    22–2910–20
    30–3320–30
    34–3730–40
    38–4040–50
    41–4350–60
    44–4560–70
    47–4970–80
    >50>80
    • The total points from table 1 are used to calculate the expected 1-year probability of death, e.g. a patient with a total risk score of 31 has a predicted 1-year probability of death of 20–30%. Reproduced with modification from [21] with permission from the publisher.

  • Table 3. The GAP (gender, age and physiology) index
    PredictorPoints
    Gender
    G Female0
     Male1
    Age years
    A ≤600
     61–651
     >652
    Physiology
     FVC % predicted
      >750
      50–751
      <502
    P DLCO % predicted
      >550
      36–551
      ≤352
      Cannot perform3
    • Maximum possible points=8. FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide.

  • Table 4. The GAP (gender, age and physiology) index and staging system
    StageIIIIII
    Points0–34–56–8
    Mortality years
     15.616.239.2
     210.929.962.1
     316.342.176.8

Additional Files

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    Files in this Data Supplement:

    • H.R. Collard
    • M. Kolb
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Staging of idiopathic pulmonary fibrosis: past, present and future
Martin Kolb, Harold R. Collard
European Respiratory Review Jun 2014, 23 (132) 220-224; DOI: 10.1183/09059180.00002114

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Staging of idiopathic pulmonary fibrosis: past, present and future
Martin Kolb, Harold R. Collard
European Respiratory Review Jun 2014, 23 (132) 220-224; DOI: 10.1183/09059180.00002114
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  • Article
    • Abstract
    • Introduction
    • The importance of staging in disease management
    • Traditional approaches to staging IPF: mild, moderate and severe
    • Limitations of the traditional approach to IPF staging
    • Novel IPF staging systems
    • Future directions
    • Conclusion
    • Acknowledgments
    • Footnotes
    • References
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