The objective of this study was to investigate predictors of survival in FA. Within a prospective registry established by the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS; identifier NCT02069509), genetically confirmed FA patients were enrolled at 11 tertiary centers and followed in yearly intervals. Overall survival was investigated applying the Kaplan-Meier method, life tables, and log-rank test. Prognostic factors were explored applying Cox proportional hazards regression and subsequently a risk score was built to assess for discrimination and calibration performance. Between September 2010 and March 2017, 631 FA patients were enrolled. Median age at inclusion was 31 (range, 6-76) years. Until December 2022, 44 patients died and 119 terminated the study for other reasons. The 10-year cumulative survival rate was 87%. In a multivariable analysis, the disability stage (hazard ratio [HR] 1.51, 95% CI 1.08-2.12, P = 0.02), history of arrhythmic disorder (HR 2.93, 95% CI 1.34-6.39, P = 0.007), and diabetes mellitus (HR 2.31, 95% CI 1.05-5.10, P = 0.04) were independent predictors of survival. GAA repeat lengths did not improve the survival model. A risk score built on the previously described factors plus the presence of left ventricular systolic dysfunction at echocardiography enabled identification of four trajectories to prognosticate up to 10-year survival (log-rank test P < 0.001). Arrhythmias, progressive neurological disability, and diabetes mellitus influence the overall survival in FA. The authors built a survival prognostic score which identifies patients meriting closer surveillance and who may benefit from early invasive cardiac monitoring and therapy.

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