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. 2005 Jul;60(7):588–594. doi: 10.1136/thx.2004.035220

Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review

J Swigris 1, W Kuschner 1, S Jacobs 1, S Wilson 1, M Gould 1
PMCID: PMC1747452  PMID: 15994268

Abstract

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.

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Selected References

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  1. Chang J. A., Curtis J. R., Patrick D. L., Raghu G. Assessment of health-related quality of life in patients with interstitial lung disease. Chest. 1999 Nov;116(5):1175–1182. doi: 10.1378/chest.116.5.1175. [DOI] [PubMed] [Google Scholar]
  2. Clark M., Cooper B., Singh S., Cooper M., Carr A., Hubbard R. A survey of nocturnal hypoxaemia and health related quality of life in patients with cryptogenic fibrosing alveolitis. Thorax. 2001 Jun;56(6):482–486. doi: 10.1136/thorax.56.6.482. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Coultas D. B., Zumwalt R. E., Black W. C., Sobonya R. E. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med. 1994 Oct;150(4):967–972. doi: 10.1164/ajrccm.150.4.7921471. [DOI] [PubMed] [Google Scholar]
  4. Counsell C. Formulating questions and locating primary studies for inclusion in systematic reviews. Ann Intern Med. 1997 Sep 1;127(5):380–387. doi: 10.7326/0003-4819-127-5-199709010-00008. [DOI] [PubMed] [Google Scholar]
  5. Curtis J. R., Deyo R. A., Hudson L. D. Pulmonary rehabilitation in chronic respiratory insufficiency. 7. Health-related quality of life among patients with chronic obstructive pulmonary disease. Thorax. 1994 Feb;49(2):162–170. doi: 10.1136/thx.49.2.162. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. De Vries J., Kessels B. L., Drent M. Quality of life of idiopathic pulmonary fibrosis patients. Eur Respir J. 2001 May;17(5):954–961. doi: 10.1183/09031936.01.17509540. [DOI] [PubMed] [Google Scholar]
  7. De Vries J., Seebregts A., Drent M. Assessing health status and quality of life in idiopathic pulmonary fibrosis: which measure should be used? Respir Med. 2000 Mar;94(3):273–278. doi: 10.1053/rmed.1999.0736. [DOI] [PubMed] [Google Scholar]
  8. Douglas W. W., Ryu J. H., Swensen S. J., Offord K. P., Schroeder D. R., Caron G. M., DeRemee R. A. Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis. A randomized prospective study. Members of the Lung Study Group. Am J Respir Crit Care Med. 1998 Jul;158(1):220–225. doi: 10.1164/ajrccm.158.1.9709089. [DOI] [PubMed] [Google Scholar]
  9. Efficace F., Bottomley A. Health related quality of life assessment methodology and reported outcomes in randomised controlled trials of primary brain cancer patients. Eur J Cancer. 2002 Sep;38(14):1824–1831. doi: 10.1016/s0959-8049(02)00173-9. [DOI] [PubMed] [Google Scholar]
  10. Efficace Fabio, Bottomley Andrew, van Andel George. Health related quality of life in prostate carcinoma patients: a systematic review of randomized controlled trials. Cancer. 2003 Jan 15;97(2):377–388. doi: 10.1002/cncr.11065. [DOI] [PubMed] [Google Scholar]
  11. Flaherty K. R., Toews G. B., Lynch J. P., 3rd, Kazerooni E. A., Gross B. H., Strawderman R. L., Hariharan K., Flint A., Martinez F. J. Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival. Am J Med. 2001 Mar;110(4):278–282. doi: 10.1016/s0002-9343(00)00711-7. [DOI] [PubMed] [Google Scholar]
  12. Guyatt G. H., Feeny D. H., Patrick D. L. Measuring health-related quality of life. Ann Intern Med. 1993 Apr 15;118(8):622–629. doi: 10.7326/0003-4819-118-8-199304150-00009. [DOI] [PubMed] [Google Scholar]
  13. Ito Kazuhiro, Ito Misako, Elliott W. Mark, Cosio Borja, Caramori Gaetano, Kon Onn Min, Barczyk Adam, Hayashi Shizu, Adcock Ian M., Hogg James C. Decreased histone deacetylase activity in chronic obstructive pulmonary disease. N Engl J Med. 2005 May 12;352(19):1967–1976. doi: 10.1056/NEJMoa041892. [DOI] [PubMed] [Google Scholar]
  14. Martinez T. Y., Pereira C. A., dos Santos M. L., Ciconelli R. M., Guimarães S. M., Martinez J. A. Evaluation of the short-form 36-item questionnaire to measure health-related quality of life in patients with idiopathic pulmonary fibrosis. Chest. 2000 Jun;117(6):1627–1632. doi: 10.1378/chest.117.6.1627. [DOI] [PubMed] [Google Scholar]
  15. McHorney C. A., Tarlov A. R. Individual-patient monitoring in clinical practice: are available health status surveys adequate? Qual Life Res. 1995 Aug;4(4):293–307. doi: 10.1007/BF01593882. [DOI] [PubMed] [Google Scholar]
  16. Meade M. O., Richardson W. S. Selecting and appraising studies for a systematic review. Ann Intern Med. 1997 Oct 1;127(7):531–537. doi: 10.7326/0003-4819-127-7-199710010-00005. [DOI] [PubMed] [Google Scholar]
  17. Nishiyama O., Taniguchi H., Kondoh Y., Kimura T., Ogawa T., Watanabe F., Nishimura K. Health-related quality of life in patients with idiopathic pulmonary fibrosis. What is the main contributing factor? Respir Med. 2005 Apr;99(4):408–414. doi: 10.1016/j.rmed.2004.09.005. [DOI] [PubMed] [Google Scholar]
  18. Panos R. J., Mortenson R. L., Niccoli S. A., King T. E., Jr Clinical deterioration in patients with idiopathic pulmonary fibrosis: causes and assessment. Am J Med. 1990 Apr;88(4):396–404. doi: 10.1016/0002-9343(90)90495-y. [DOI] [PubMed] [Google Scholar]
  19. Raghu Ganesh, Brown Kevin K., Bradford Williamson Z., Starko Karen, Noble Paul W., Schwartz David A., King Talmadge E., Jr, Idiopathic Pulmonary Fibrosis Study Group A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2004 Jan 8;350(2):125–133. doi: 10.1056/NEJMoa030511. [DOI] [PubMed] [Google Scholar]
  20. Ramsey S. D., Patrick D. L., Lewis S., Albert R. K., Raghu G. Improvement in quality of life after lung transplantation: a preliminary study. The University of Washington Medical Center Lung Transplant Study Group. J Heart Lung Transplant. 1995 Sep-Oct;14(5):870–877. [PubMed] [Google Scholar]
  21. Spencer S., Calverley P. M., Sherwood Burge P., Jones P. W., ISOLDE Study Group. Inhaled Steroids in Obstructive Lung Disease Health status deterioration in patients with chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2001 Jan;163(1):122–128. doi: 10.1164/ajrccm.163.1.2005009. [DOI] [PubMed] [Google Scholar]
  22. Vincken W., van Noord J. A., Greefhorst A. P. M., Bantje Th A., Kesten S., Korducki L., Cornelissen P. J. G., Dutch/Belgian Tiotropium Study Group Improved health outcomes in patients with COPD during 1 yr's treatment with tiotropium. Eur Respir J. 2002 Feb;19(2):209–216. doi: 10.1183/09031936.02.00238702. [DOI] [PubMed] [Google Scholar]

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