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Title: Disease progression in idiopathic pulmonary fibrosis with mild physiological impairment: analysis from the Australian IPF registry.
Austin Authors: Jo, Helen E;Glaspole, Ian;Moodley, Yuben;Chapman, Sally;Ellis, Samantha;Goh, Nicole S L ;Hopkins, Peter;Keir, Greg;Mahar, Annabelle;Cooper, Wendy;Reynolds, Paul;Haydn Walters, E;Zappala, Christopher;Grainge, Christopher;Allan, Heather;Macansh, Sacha;Corte, Tamera J
Affiliation: Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW, 2050, Australia
Royal Brisbane and Women's Hospital, Herston, QLD, Australia
University of Sydney, Camperdown, NSW, Australia
John Hunter Hospital, New Lambton Heights, NSW, Australia
Lung Foundation Australia, Milton, QLD, Australia
The Alfred Hospital, Melbourne, Victoria, Australia
Fiona Stanley Hospital, Murdoch, WA, Australia
Royal Adelaide Hospital, Adelaide, SA, Australia
Austin Health
Prince Charles Hospital, Chermside West, QLD, Australia
Princess Alexandra Hospital, Woolloongabba, QLD, Australia
School of Medicine, Western Sydney University, Parramatta, NSW, Australia
School of Medicine, University of Tasmania, Hobart, TAS, Australia
University of Tasmania, Hobart, TAS, Australia
Issue Date: 25-Jan-2018
Date: 2018
Publication information: BMC Pulmonary Medicine 2018-01-25; 18(1): 19
Abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal fibrosing lung disease of unknown cause. The advent of anti-fibrotic medications known to slow disease progression has revolutionised IPF management in recent years. However, little is known about the natural history of IPF patients with mild physiological impairment. We aimed to assess the natural history of these patients using data from the Australian IPF Registry (AIPFR). Using our cohort of real-world IPF patients, we compared FVC criteria for mild physiological impairment (FVC ≥ 80%) against other proposed criteria: DLco ≥ 55%; CPI ≤40 and GAP stage 1 with regards agreement in classification and relationship with disease outcomes. Within the mild cohort (FVC ≥ 80%), we also explored markers associated with poorer prognosis at 12 months. Of the 416 AIPFR patients (mean age 70.4 years, 70% male), 216 (52%) were classified as 'mild' using FVC ≥ 80%. There was only modest agreement between FVC and DLco (k = 0.30), with better agreement with GAP (k = 0.50) and CPI (k = 0.48). Patients who were mild had longer survival, regardless of how mild physiologic impairment was defined. There was, however, no difference in the annual decline in FVC% predicted between mild and moderate-severe groups (for all proposed criteria). For patients with mild impairment (n = 216, FVC ≥ 80%), the strongest predictor of outcomes at 12 months was oxygen desaturation on a 6 min walk test. IPF patients with mild physiological impairment have better survival than patients with moderate-severe disease. Their overall rate of disease progression however, is comparable, suggesting that they are simply at different points in the natural history of IPF disease.
DOI: 10.1186/s12890-018-0575-y
ORCID: 0000-0003-1183-2729
Journal: BMC Pulmonary Medicine
PubMed URL: 29370786
PubMed URL:
Type: Journal Article
Subjects: Disease severity
Idiopathic pulmonary fibrosis
Interstitial lung disease
Appears in Collections:Journal articles

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