RT Journal Article
SR Electronic
T1 Patient reported distress can aid clinical decision making in idiopathic pulmonary fibrosis: analysis of the PROFILE cohort
JF bioRxiv
FD Cold Spring Harbor Laboratory
SP 460626
DO 10.1101/460626
A1 Iain Stewart
A1 Tricia McKeever
A1 Rebecca Braybrooke
A1 Eunice Oballa
A1 Juliet K Simpson
A1 Toby M Maher
A1 Richard P Marshall
A1 Pauline T Lukey
A1 William A Fahy
A1 Gisli Jenkins
A1 Gauri Saini
YR 2018
UL http://biorxiv.org/content/early/2018/12/13/460626.abstract
AB 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.