TY - JOUR T1 - Development and External Validation of 1- and 2- year Mortality Prediction Models in Cystic Fibrosis JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00224-2019 SP - 1900224 AU - Sanja Stanojevic AU - Jenna Sykes AU - Anne L. Stephenson AU - Shawn D. Aaron AU - G.A. Whitmore Y1 - 2019/01/01 UR - http://erj.ersjournals.com/content/early/2019/05/08/13993003.00224-2019.abstract N2 - Introduction We aimed to develop a clinical tool for predicting 1- and 2-year risk of death for patients with cystic fibrosis (CF). The model considers patients' overall health status as well as risk of intermittent shock events in calculating the risk of death.Methods Canadian CF Registry data from 1982 to 2015 were used to develop a predictive risk model using threshold regression. A 2-year risk of death estimated conditional probability of surviving the second year given survival for the first year. UK CF Registry data from 2008 to 2013 were used to externally validate the model.Results The combined effect of CF chronic health status and CF intermittent shock risk provided a simple clinical scoring tool for assessing 1-year and 2-year risk of death for an individual CF patient. At a threshold risk of death of 20% or greater, the one-year model had a sensitivity of 74% and specificity of 96%. The area under the receiver operating curve (AUC) for the 2-year mortality model was significantly greater than the AUC for a model that predicted survival based on FEV1 below 30% predicted (AUC 0.95 versus 0.68 respectively, p<0.001). The Canadian-derived model validated well with the UK data and correctly identified 79% of deaths and 95% of survivors in a single year in the UK.Conclusions The prediction models provide an accurate risk of death over a one and two-year time horizon. The models performed equally well when validated in an independent UK CF population.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Stanojevic has nothing to disclose.Conflict of interest: Ms Sykes has nothing to disclose.Conflict of interest: Dr. Stephenson has nothing to disclose.Conflict of interest: Dr. Aaron has nothing to disclose.Conflict of interest: Dr. Whitmore has nothing to disclose. ER -