Mortality risk prediction in amyopathic dermatomyositis associated with interstitial lung disease: the FLAIR model.

Abstract Background The prognosis of amyopathic dermatomyositis (ADM)-associated interstitial lung disease (ILD) is poor. A mortality risk score model is needed to predict survival in patients with ADM-ILD and guide clinical treatment. Research Question How to identify ADM-ILD patients at high risk and to predict patients' outcome based on a risk stratification model? Study Design and Methods: We evaluated 207 patients with ADM-ILD in this prospective inception study. We used a multivariable Cox proportional hazards model to identify the independent prognostic risk factors and created a risk score model according to patients' data from January 2012 to December 2016. We used the index of prediction accuracy (IPA) which utilizes the Brier score to reflect both discrimination and calibration of the model. The model was validated in an independent group of patients from January 2017 to June 2018. Results We developed a combined risk score, the FLAIR score that included the following values and scores: ferritin ( Interpretation The FLAIR risk score model could help predict survival in ADM-ILD patients and guide further clinical research on risk-based treatment.