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Patient reported distress can aid clinical decision making in idiopathic pulmonary fibrosis: analysis of the PROFILE cohort

Stewart, Iain; McKeever, Tricia; Braybrooke, Rebecca; Oballa, Eunice; Simpson, Juliet K.; Maher, Toby M.; Marshall, Richard P.; Lukey, Pauline T.; Fahy, William A.; Jenkins, Gisli; Saini, Gauri


Iain Stewart

Professor of Epidemiology and Medical Statistics

Rebecca Braybrooke

Eunice Oballa

Juliet K. Simpson

Toby M. Maher

Richard P. Marshall

Pauline T. Lukey

William A. Fahy

Gisli Jenkins

Gauri Saini


Idiopathic pulmonary fibrosis is a progressive and fatal interstitial lung disease. We aimed to determine if patient response to a palliative assessment survey could predict disease progression or death.

We undertook a cross-sectional study in a UK clinical cohort of incident cases. Rasch-based methodology provided a disease distress value from an abridged 11 item model of the original 45 item survey. Distress values were compared with measures of lung function. Disease progression or mortality alone was predicted at twelve months from survey completion, with risk of death assessed at three, six and twelve months.

Disease distress values were negatively correlated with lung function (r=-0.275 percent predicted DLCO). Expected survey scores computed from distress values could distinguish disease progression, 8.8 (p=0.004), and people who died, 10.2 (p=0.002), from those who did not progress, 6.9. Actual survey scores predicted disease progression and mortality with an area under the curve of 0.60 and 0.64, respectively. Each point increment in actual score increased risk of twelve-month mortality by 10%, almost 43% of people scoring above 18 did not survive beyond 105 days.

We define a short questionnaire that can score disease distress and predict prognosis, assisting clinical decision making in progressive fibrosis.


Stewart, I., McKeever, T., Braybrooke, R., Oballa, E., Simpson, J. K., Maher, T. M., …Saini, G. (2019). Patient reported distress can aid clinical decision making in idiopathic pulmonary fibrosis: analysis of the PROFILE cohort. European Respiratory Journal, 53(5), Article 1801925.

Journal Article Type Article
Acceptance Date Feb 13, 2019
Online Publication Date Mar 7, 2019
Publication Date May 1, 2019
Deposit Date Feb 28, 2019
Publicly Available Date Sep 8, 2020
Journal European Respiratory Journal
Print ISSN 0903-1936
Electronic ISSN 1399-3003
Publisher European Respiratory Society
Peer Reviewed Peer Reviewed
Volume 53
Issue 5
Article Number 1801925
Public URL
Publisher URL
Additional Information This is an author-submitted, peer-reviewed version of a manuscript that has been accepted for publication in the European Respiratory Journal, prior to copy-editing, formatting and typesetting. This version of the manuscript may not be duplicated or reproduced without prior permission from the copyright owner, the European Respiratory Society. The publisher is not responsible or liable for any errors or omissions in this version of the manuscript or in any version derived from it by any other parties. The final, copy-edited, published article, which is the version of record, is available without a subscription 18 months after the date of issue publication


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