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Disease progression in early idiopathic pulmonary fibrosis: Insights from the Australian IPF registry

Helen Jo, Ian Glaspole, Yuben Moodley, Sally Chapman, Wendy Cooper, Samantha Ellis, Nicole Goh, Peter Hopkins, Greg Keir, Annabelle Mahar, Paul Reynolds, Haydn Walters, Christopher Zappala, Christopher Grainge, Heather Allan, Tamera Corte
European Respiratory Journal 2016 48: PA2100; DOI: 10.1183/13993003.congress-2016.PA2100
Helen Jo
Respiratory, Royal Prince Alfred, Sydney, NSWAustraliaMedicine, University of Sydney, Sydney, NSWAustralia
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Ian Glaspole
Respiratory, The Alfred, Melbourne, VICAustralia
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Yuben Moodley
Respiratory, Fiona Stanley Hospital, Perth, WAAustralia
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Sally Chapman
Respiratory, Royal Adelaide Hospital, Adelaide, Australia
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Wendy Cooper
Respiratory, Royal Prince Alfred, Sydney, NSWAustralia
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Samantha Ellis
Respiratory, The Alfred, Melbourne, VICAustralia
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Nicole Goh
Respiratory, The Alfred, Melbourne, VICAustralia
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Peter Hopkins
Respiratory, Prince Charles Hospital, Brisbane, Australia
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Greg Keir
Respiratory, Princess Alexandria Hospital, Brisbane, Australia
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Annabelle Mahar
Respiratory, Royal Prince Alfred, Sydney, NSWAustralia
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Paul Reynolds
Respiratory, Royal Adelaide Hospital, Adelaide, Australia
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Haydn Walters
CRE, University of Tasmania, Hobart, Australia
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Christopher Zappala
Respiratory, Royal Brisbane and Women's Hospital, Brisbane, Australia
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Christopher Grainge
Respiratory, John Hunter Hospital, Newcastle, Australia
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Heather Allan
Lung Foundation, Lung Foundation Australia, Brisbane, Australia
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Tamera Corte
Respiratory, Royal Prince Alfred, Sydney, NSWAustraliaMedicine, University of Sydney, Sydney, NSWAustralia
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Abstract

Idiopathic pulmonary fibrosis (IPF) is progressive with a median survival of 2-3yrs. The natural history is highly variable and difficult to predict for an individual presenting with early IPF.

Aim: To identify markers associated with disease progression for IPF patients; especially those with early disease.

Method: The Australian IPF Registry recruits IPF patients across Australia collating clinical, physiological and questionnaire data. Using this real-world longitudinal cohort we compared mild disease(FVC >80%) with FVC<80%. Using Cox analysis and logistic regression,we examined associations between pre-specified markers and progression free survival(PFS;fall in FVC>10% or DLco>15% ± death).

Result: Baseline data in 631 participants revealed: 433(69%) male, mean age 70.1±8.5yrs; mean FVC 81±22% and DLco 49±17%. Patients with mild disease(n=235) were older(p=0.001) and more often female(p<0.001), than those with FVC<80%.

Reduced PFS was associated with: male gender, impaired quality of life(SGRQ), depression, cough severity, and lower baseline FVC and DLco. Patients with mild disease had improved PFS compared to those with FVC<80%(HR 1.61;95% CI1.3,2.0;P<0.001).

In the subset of patients with mild disease, 41 (18%) had disease progression at 12mths. In mild IPF, disease progression at 12mths was associated with: 6MWT end SpO2 (OR 0.91, 95%CI 0.84-0.99; p=0.028), 6MWT distance and cough severity. On multivariate regression, the variable most closely associated with death at 12mths was 6MWT end SpO2.

Conclusion: Impaired baseline lung function is associated with a worse PFS in IPF patients. However a significant proportion of patients with mild disease also show disease progression at 12mths.

  • Idiopathic pulmonary fibrosis
  • Interstitial lung disease
  • Copyright ©the authors 2016
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Disease progression in early idiopathic pulmonary fibrosis: Insights from the Australian IPF registry
Helen Jo, Ian Glaspole, Yuben Moodley, Sally Chapman, Wendy Cooper, Samantha Ellis, Nicole Goh, Peter Hopkins, Greg Keir, Annabelle Mahar, Paul Reynolds, Haydn Walters, Christopher Zappala, Christopher Grainge, Heather Allan, Tamera Corte
European Respiratory Journal Sep 2016, 48 (suppl 60) PA2100; DOI: 10.1183/13993003.congress-2016.PA2100

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Disease progression in early idiopathic pulmonary fibrosis: Insights from the Australian IPF registry
Helen Jo, Ian Glaspole, Yuben Moodley, Sally Chapman, Wendy Cooper, Samantha Ellis, Nicole Goh, Peter Hopkins, Greg Keir, Annabelle Mahar, Paul Reynolds, Haydn Walters, Christopher Zappala, Christopher Grainge, Heather Allan, Tamera Corte
European Respiratory Journal Sep 2016, 48 (suppl 60) PA2100; DOI: 10.1183/13993003.congress-2016.PA2100
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