Health-related quality of life in idiopathic pulmonary fibrosis: Data from the Australian IPF Registry.
Studies analysing the effect of worsening pulmonary physiological impairment in idiopathic pulmonary fibrosis (IPF) with respect to quality of life have been limited to single centres or highly selected trial populations. The aim of this study was to determine the principal determinants of baseline and longitudinal health-related quality of life (HRQoL) in a large unselected IPF population.



We used the Australian IPF Registry to examine the relationship between HRQoL, measured using the St George Respiratory Questionnaire (SGRQ), and demographic features, physiological features, co-morbidities and symptoms. Linear regression analysis was performed to identify predictors of baseline HRQoL, linear mixed model analysis to determine the effect of time and forced vital capacity (FVC) on SGRQ and Cox proportional hazards regression to examine the relationship between HRQoL and all-cause mortality.



Baseline data from 516 patients were available (347 males; mean (SD) age: 71.3 ± 8.6 years). Univariate analysis showed significant associations between HRQoL and demographic, clinical and physiological features. However, multivariate analysis demonstrated independent associations only between SGRQ and dyspnoea (University of California San Diego Shortness of Breathlessness Questionnaire (UCSD-SOBQ); R2= 0.71, P < 0.0001), cough severity (visual analogue scale; R2= 0.06, P < 0.0001) and depression (Hospital Anxiety and Depression Scale; R2= 0.04, P < 0.0001). Linear mixed-effects modelling of combined baseline and longitudinal data confirmed these associations, as well as for FVC% predicted (P = 0.005). Multivariate Cox proportionate-proportional hazards regression analysis demonstrated no association between HRQoL and risk of mortality.



Cough, dyspnoea and depression are major symptomatic determinants of HRQoL in IPF. FVC decline is associated with worsening HRQoL.
Department of Allergy, Immunology and Respiratory Medicine, The Alfred Hospital, Melbourne, Victoria, Australia.|Department of Medicine, Monash University, Melbourne, Victoria, Australia.
Ian N
0000-0002-5118-2890
IN
Glaspole
Department of Medicine, Royal Adelaide Hospital, Adelaide, South Australia, Australia.
Sally A
SA
Chapman
Department of Anatomical Pathology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.|Department of Pathology, University of Sydney, Sydney, New South Wales, Australia.|School of Medicine, University of Western Sydney, Sydney, New South Wales, Australia.
Wendy A
WA
Cooper
Department of Radiology, The Alfred Hospital, Melbourne, Victoria, Australia.
Samantha J
SJ
Ellis
Department of Allergy, Immunology and Respiratory Medicine, The Alfred Hospital, Melbourne, Victoria, Australia.|Institute of Breathing and Sleep, Austin Hospital, Melbourne, Victoria, Australia.
Nicole S
NS
Goh
Department of Thoracic Medicine, Prince Charles Hospital, Brisbane, Queensland, Australia.
Peter M
PM
Hopkins
Australian IPF Registry, Lung Foundation of Australia, Brisbane, Queensland, Australia.
Sacha
S
Macansh
Department of Anatomical Pathology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.|Department of Pathology, University of Sydney, Sydney, New South Wales, Australia.
Annabelle
A
Mahar
Department of Respiratory Medicine, Fiona Stanley Hospital, Perth, Western Australia, Australia.
Yuben P
YP
Moodley
Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, Victoria, Australia.|Clinical Haematology Department, The Alfred Hospital, Melbourne, Victoria, Australia.
Eldho
E
Paul
Department of Medicine, Royal Adelaide Hospital, Adelaide, South Australia, Australia.
Paul N
PN
Reynolds
NHMRC CRE for Chronic Respiratory Disease, University of Tasmania, Hobart, Tasmania, Australia.
E Haydn
EH
Walters
Royal Brisbane & Women's Hospital, Brisbane, Queensland, Australia.
Christopher J
CJ
Zappala
Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.|Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
Tamera J
TJ
Corte
D000368:Aged; D001315:Australia; D003371:Cough / Q000209:etiology; D003863:Depression; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000503:physiopathology* / Q000523:psychology; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011788:Quality of Life; D012042:Registries; D011795:Surveys and Questionnaires; D014797:Vital Capacity
cough; dyspnoea; forced vital capacity; pulmonary fibrosis; quality of life