Article Text

Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review
  1. J J Swigris1,
  2. W G Kuschner1,2,
  3. S S Jacobs1,
  4. S R Wilson1,3,
  5. M K Gould1,2
  1. 1Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, CA, USA
  2. 2VA Palo Alto Health Care System, Palo Alto, CA, USA
  3. 3Palo Alto Medical Foundation Research Institute, Palo Alto Medical Foundation, Palo Alto, CA, USA
  1. Correspondence to:
    Dr J J Swigris
    Stanford University Medical Center, Division of Pulmonary and Critical Care Medicine, MC5236, Room H3143, 300 Pasteur Drive, Stanford, CA 94305-5236, USA;


Background: Idiopathic pulmonary fibrosis (IPF) profoundly affects the quality of patients’ lives. A systematic review was performed to evaluate critically the published literature and to examine what is known about health-related quality of life (HRQL) in patients with IPF.

Methods: The MEDLINE, EMBASE, Health and Psychosocial Instruments, and Cochrane Library databases were searched to 1 April 2004. Abstracts and bibliographies of published articles were scanned and contact was made with investigators. Included studies analysed HRQL (or quality of life) in at least 10 patients with IPF. Two reviewers independently selected studies, evaluated their quality according to predetermined criteria, and abstracted data on study design, patients’ demographic and clinical characteristics, and quality of life outcome measures.

Results: Seven studies met the inclusion criteria. The studies enrolled 512 patients with IPF and used three different instruments to measure HRQL. All studies had important limitations in methodological quality; none measured longitudinal changes in HRQL over time. Patients reported substantially impaired HRQL, especially in domains that measured physical health and level of independence. Patients with IPF appear to have similar impairments in HRQL to those with chronic obstructive pulmonary disease. Measures of dyspnoea were moderately correlated with scores from domains that measured physical health (R2 = 0.03–0.66) and energy/fatigue/pep (R2 = 0.19–0.55), but measures of pulmonary function and gas exchange did not correlate as strongly with these and other domains.

Conclusion: Studies of HRQL in patients with IPF suggest that, in addition to the obvious effect on physical health, general health, energy level, respiratory symptoms, and level of independence are also impaired. Variability in HRQL among patients is not fully explained by measures of dyspnoea or pulmonary function, suggesting that HRQL measures provide unique information. More research is needed to identify or design appropriate measurement instruments for patients with IPF and to examine changes in HRQL over time or in response to specific treatments.

  • FVC, forced vital capacity
  • HQOL, health-related quality of life
  • IPF, idiopathic pulmonary fibrosis
  • Pao2, arterial oxygen tension
  • QOL, quality of life
  • SF-36, Medical Outcomes Study Short Form-36
  • SGRQ, St George’s Respiratory Questionnaire
  • TLC, total lung capacity
  • Tlco, carbon monoxide transfer factor
  • UIP, usual interstitial pneumonia
  • WHOQOL-100, WHO Quality of Life 100 Item Instrument
  • pulmonary fibrosis
  • interstitial lung disease
  • quality of life
View Full Text

Statistics from

Supplementary materials

  • The appendices are available as a downloadable PDF (printer friendly file).

    If you do not have Adobe Reader installed on your computer,
    you can download this free-of-charge, please Click here


    Files in this Data Supplement:


  • Dr Swigris received support from an NIH training grant (T32 HL07948-01A1). Dr Gould received an Advanced Research Career Development Award from the VA Health Services Research and Development Service. The views expressed in this article are those of the authors and not necessarily the views of the Department of Veterans Affairs.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.