Abstract
The objective of the present study was to determine if there is a health-related quality of life (HRQL) instrument, generic or specific, that better represents functional capacity dysfunction in idiopathic pulmonary fibrosis (IPF) patients. HRQL was evaluated in 20 IPF patients using generic and specific questionnaires (Medical Outcomes Short Form 36 (SF-36) and Saint George's Respiratory Questionnaire (SGRQ), respectively). Functional status was evaluated by pulmonary function tests, 6-min walking distance test (6MWDT) and dyspnea indexes (baseline dyspnea index) at rest and after exercise (modified Borg scale). There was a restrictive pattern with impairment of diffusion capacity (total lung capacity, TLC = 71.5 +/- 15.6%, forced vital capacity = 70.4 +/- 19.4%, and carbon monoxide diffusing capacity = 41.5 +/- 16.2% of predicted value), a reduction in exercise capacity (6MWDT = 435.6 +/- 95.5 m) and an increase of perceived dyspnea score at rest and during exercise (6 +/- 2.5 and 7.1 +/- 1.3, respectively). Both questionnaires presented correlation with some functional parameters (TLC, forced expiratory volume in 1 s and carbon monoxide diffusing capacity) and the best correlation was with TLC. Almost all of the SGRQ domains presented a strong correlation with functional status, while in SF-36 only physical function and vitality presented a good correlation with functional status. Dyspnea index at rest and 6MWDT also presented a good correlation with HRQL. Our results suggest that a specific instead of a generic questionnaire is a more appropriate instrument for HRQL evaluation in IPF patients and that TLC is the functional parameter showing best correlation with HRQL.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause associated with a surgical lung biopsy showing a histological pattern of usual interstitial pneumonia [1]
Patients presented on average impairment of exercise capacity (6MWDT average of 435.6 m) and results ranged from no limitation (648 m) to severe limitation (272 m; Table 1)
The present results showed that generic (SF-36) and specific (SGRQ) questionnaires revealed a similar decrease in healthrelated quality of life (HRQL) aspects of IPF patients
Summary
Idiopathic pulmonary fibrosis (IPF) is a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause associated with a surgical lung biopsy showing a histological pattern of usual interstitial pneumonia [1]. The age at onset is more than 50 years and IPF is slightly more common in males. It is characterized by dyspnea, nonproductive cough, reduced lung volumes, and abnormal gas exchange. Constitutional symptoms such as weight loss, malaise, and fatigue may be present [2, 3]. IPF patients present a progressive course that results in severe disability and death in the majority of cases. During the clinical course of the disease, patients invariably become severely limited in their activities and dependent on supplemental oxygen [3]
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