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

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 12 months from survey completion, with risk of death assessed at 3, 6 and 12 months.Disease distress values were negatively correlated with lung function (r=-0.275 for the percentage predicted diffusing capacity of the lung for carbon monoxide). Expected survey scores computed from distress values could distinguish disease progression (n=8.8, p=0.004) and death (n=10.2, p=0.002) from no disease progression (n=6.9). Actual survey scores predicted disease progression and death with an area under the curve of 0.60 and 0.64, respectively. Each point increment in actual score increased risk of 12-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, thus assisting clinical decision-making in progressive fibrosis.
Competing Interests: Conflict of interest: I. Stewart has nothing to disclose. Conflict of interest: T. McKeever has nothing to disclose. Conflict of interest: R. Braybrooke has nothing to disclose. Conflict of interest: E. Oballa is an employee of GlaxoSmithKline. Conflict of interest: J.K. Simpson is an employee of and shareholder in GlaxoSmithKline. Conflict of interest: T.M. Maher has, via his institution, received industry–academic funding from GlaxoSmithKline R&D and UCB, and has received consultancy or speakers fees from Apellis, AstraZeneca, Bayer, Biogen Idec, Boehringer Ingelheim, Galapagos, GlaxoSmithKline R&D, Indalo, Pliant, ProMetic, Roche, Samumed and UCB. Conflict of interest: R.P. Marshall was a paid employee of GlaxoSmithKline when the work was carried out and remains a shareholder in GlaxoSmithKline. Conflict of interest: P.T. Lukey reports that during the PROFILE study, she was an employee of, and still owns shares in, GlaxoSmithKline. For the last 3 years, she has worked, and in some cases is still working, as an independent consultant to GlaxoSmithKline R&D, the Francis Crick Institute, Syncona, Peptinnovate, Mereo BioPharma, LiFT BioScience, DJS antibodies, BergenBio and Galecto. Conflict of interest: W.A. Fahy is an employee of and shareholder in GlaxoSmithKline. Conflict of interest: G. Jenkins reports grants from GlaxoSmithKline during the conduct of the study; grants from Biogen and Galecto, personal fees from Boehringer Ingelheim, Galapagos, Heptares, Pliant and Roche, grants and personal fees from GlaxoSmithKline and MedImmune, and service on advisory boards (no fees received) for NuMedii and Redex, outside the submitted work; and is a trustee of the British Thoracic Society and Action for Pulmonary Fibrosis. Conflict of interest: G. Saini has nothing to disclose.
(Copyright ©ERS 2019.)