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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.

Introduction: The conclusion of previous investigations that pulmonary rehabilitation (PR) is an effective intervention for the management of chronic lung disease may not be generalizable to PR programs with limited experience delivering this complex, interdisciplinary service., Objective: Determine whether PR is effective for the first group of patients treated in a newly formed interdisciplinary PR program., Methods: We conducted a longitudinal analysis of changes in health-related quality of life and 6-minute walk test for the first group of patients completing our newly formed 8-week outpatient PR program. We studied 6 men, age 65-77 years, with stable severe chronic obstructive pulmonary disease. Patients completed the Chronic Respiratory Disease Questionnaire immediately before and 1 year after participation in our PR program., Results: Four patients completed the PR 6-minute walk test both before and after the program. We found improvement in all Chronic Respiratory Disease Questionnaire domains at follow-up (mean +/- SD before and after): dyspnea 1.67 +/- 0.82 vs 4.92 +/- 0.49; emotional function 2.33 +/- 0.82 vs 5.50 +/- 0.55; fatigue 2.00 +/- 0.63 vs 5.00 +/- 0.63; feeling of mastery over disease 1.83 +/- 0.41 vs 5.83 +/- 1.17. The interval improvements in all health-related quality of life domains were statistically significant (p < 0.02 for all comparisons). There was a trend toward improvement in exercise tolerance: 231 +/- 213 ft before PR vs 353 +/- 66 ft at the 1-year follow-up (p = 0.2)., Conclusions: PR can result in sustained improvement in the quality of life of patients with severe chronic obstructive pulmonary disease, even when this complex, interdisciplinary service is delivered by a newly formed and inexperienced PR program.