Health related quality of life (HRQoL) and lung function in patients with Cryptogenic Fibrosing Alveolitis (CFA)

Introduction: The prognosis for patients with CFA is poor and symptoms are often marked. In order to assess the efficacy of interventions in this patient group we need suitable measures of HRQoL. We have therefore undertaken a survey of lung function and HRQoL in a population of patients with CFA attending one centre. Methods: We attempted to identify all patients with CFA attending Nottingham City Hospital using a combination of lung function records, in-patient records and local diagnostic databases. Cases were confirmed if our established diagnostic criteria were met and we included patients with connective tissue diseases. Each patient attended for spirometry, capillary blood gases and to complete four questionnaires: Guyatt's McMaster Chronic Respiratory Disease Questionnaire (CRDQ), Short Form-36 Health Survey (SF-36), Hospital Anxiety and Depression score (HAD) and the Schedule for the Evaluation of Individualised Quality of Life (SEIQoL). We then assessed the correlation between physiological measures (percent predicted FVC (%FVC) and resting PaO2) and HRQoL measures adjusting for the effects of age and sex. Results: Of 67 patients identified, 49 patients took part; 11 refused, 2 did not reply and 5 were too ill to come to hospital. There were 32 males (66%), the mean age was 68 years. Mean percent predicted FVC was 87% and mean PaO2 was 9.2kPa. We found evidence of correlation between %FVC and the mastery domain of the CRDQ (p=0.02), the depression score of HAD (0.001), and the physical function (p=0.03), energy/vitality (p=0.018) and general health perception (p=0.001) domains of SF-36. A similar pattern was found for correlations between PaO2 and HRQol. Conclusions: In this cross-sectional study elements of the CRDQ, HAD and SF-36 questionnaires were all correlated to some degree with physiological measurements in patients with CFA.