The Risk Stratification Score, a New Staging System for Idiopathic Pulmonary Fibrosis: Final Data of the Discovery Cohort

Abstract RATIONALE Idiopathic pulmonary fibrosis (IPF) is characterized by an unfavorable prognosis and highly variable rates of progression. A new staging system that is reliably responsive to longitudinal changes in the individual patient is required to guide clinical decision making (including lung transplant listing). Using a multidimensional approach, we conducted an observational, prospective, multicentre study with longitudinal visits at 4 months intervals, and including both a discovery and a validation cohort. The proposed RIsk Stratification scorE (RISE) is a 4-point index combining MRC dyspnea score (MRCDS), 6-minute walking distance (6MWD) and the Composite Physiologic Index (CPI; CALIPER-revised). The aim of this interim analysis was to investigate the prognostic performance of RISE in the discovery cohort. METHODS We conducted a preliminary analysis of the discovery cohort. Newly diagnosed patients with IPF were include in the study and followed for a period of the 3-years (ClinicalTrials.gov trial n. NCT02632123) at 2 interstitial lung disease centres (London, Canada and Rome, Italy). The primary endpoint was lung transplant (LTx)-free survival. RESULTS Among 179 patients included in the discovery cohort, there were 71 deaths, and 16 patients received a LTx (combined mortality/LTx 48.6%)). Multi-variate Cox regression analysis showed that the 3 individual variables included in the study (namely MRCDS ≥3, 6MWD ≤78% predicted and CPI >23) independently and significantly predicted 3-year LTx free survival, while the receiver operating characteristic (ROC) analysis demonstrated very good diagnostic accuracy for the prediction of the endpoint, with an area under the curve (AUC) of 0.775, 0.713 and 0.868, respectively. RISE was calculated based on the 3 parameters. The Kaplan-Meier curves and the log-rank test showed a very good predictive power of baseline RISE and an excellent predictive power for its longitudinal changes (Chi square 67.04 and 80.74, respectively). CONCLUSIONS This preliminary analysis of the discovery cohort shows that the individual components included in the score are independent predictors of LTx-free survival, which validates the multidimensional approach based on the use of variables belonging to different domains (dyspnea, lung function, exercise capacity). The analysis of the discovery cohort also showed that the RISE and is responsive to longitudinal changes observed during the clinical course, making it a reliable and accurate staging system. We look forward to confirming these results in the ongoing validation cohort.

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