Research Article: Development of a predictive tool for hemophagocytic lymphohistiocytosis/macrophage activation syndrome risk in adult-onset Still’s disease
Abstract:
Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) is one of the most common complications of adult-onset Still’s disease (AOSD), and a leading cause of organ failure and mortality in these patients. Early diagnosis and management are crucial but can be challenging. Our study aims to develop a tool for predicting the risk of HLH/MAS in AOSD patients.
The study recruited AOSD patients admitted to Ruijin Hospital from May 2018 to May 2024. Clinical features and laboratory test results were collected within the first 3 days of hospitalization. Predictor variables were selected using LASSO regression, and a logistic regression model was subsequently developed to predict HLH/MAS. Model performance was assessed through calibration curves, receiver operating characteristic (ROC) curve, decision curve analysis (DCA), and bootstrap internal validation.
This retrospective cohort study included 190 patients with AOSD. Those who developed HLH/MAS had a higher incidence of fever and splenomegaly at admission. They also exhibited more significant reductions in blood cell counts, elevated inflammatory markers, liver dysfunction, and coagulopathy compared to patients without HLH/MAS. We developed the HLH/MAS Risk Score, which included five key predictors—body temperature, platelet count, D-dimer levels, systemic score, and initial glucocorticoid response—to assess the HLH/MAS risk in hospitalized AOSD patients. This model was visualized and demonstrated robust predictive performance, achieving an AUC of 0.86. Comparative analysis with other MAS-related models confirmed that the HLH/MAS Risk Score had competitive discriminative power.
In this study, we developed the HLH/MAS Risk Score, a predictive tool designed to assess the risk of HLH/MAS in AOSD patients during hospitalization. The model demonstrated strong predictive performance and provides a reliable, easy-to-use tool for the early identification of patients at high risk for HLH/MAS. Further external validation is needed to confirm its clinical applicability across diverse populations.
Introduction:
Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) is one of the most common complications of adult-onset Still’s disease (AOSD), and a leading cause of organ failure and mortality in these patients. Early diagnosis and management are crucial but can be challenging. Our study aims to develop a tool for predicting the risk of HLH/MAS in AOSD patients.
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