Development and Validation of the <scp>ALARN</scp> Model: A Predictive Mortality Score for Antisynthetase Syndrome

Objectives To develop and validate a prognostic model for predicting all‐cause mortality in patients with antisynthetase syndrome (ASyS). Methods This retrospective study included 1194 patients with ASyS from three independent institutions in China. The Cox proportional hazards (CPH) method and random survival forest (RSF) algorithm were used for developing a mortality risk prediction model in derivation cohort (n=763). The optimal model was simplified into a scoring system and validated in an external cohort of 431 patients from two institutions. Results The model constructed using the CPH method in the training cohort incorporated five prognostic factors: age at onset, lactate dehydrogenase, albumin, respiratory failure, and neutrophil‐to‐lymphocyte ratio. This model demonstrated better performance than the model developed using the RSF algorithm in the internal validation cohort and was subsequently transformed into a simplified scoring system, termed the ALARN score. The ALARN model achieved time‐dependent areas under the receiver operating characteristic curves of 0.914 (95% CI: 0.866–0.955), 0.867 (95% CI: 0.829–0.906), 0.839 (95% CI: 0.789–0.885), and 0.854 (95% CI: 0.789–0.909) for predicting 1‐, 3‐, 5‐, and 10‐year mortality, respectively. Moreover, patients were effectively stratified into low‐, intermediate‐, and high‐risk groups (ALARN scores of 0–2, 3–5, and 6–8, respectively) with significantly different survival outcomes (log‐rank p&lt;0.0001). Internal and external validation confirmed the robustness of the ALARN score in predicting overall mortality in ASyS. Conclusions The ALARN score incorporates five readily available clinical parameters and provides a simple, practical tool for predicting mortality risk in patients with ASyS.

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Journal
Arthritis & Rheumatology
Published
2026-06-09
DOI
https://doi.org/10.1002/art.70249
Primary Topic
Inflammatory Myopathies and Dermatomyositis
Type
article
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article

Development and Validation of the ALARN Model: A Predictive Mortality Score for Antisynthetase Syndrome

Chen Zong, G C Wang, Yiran Chen, Yingfang Zhang et al.
Arthritis & Rheumatology
Inflammatory Myopathies and Dermatomyositis
article

Development and Validation of the ALARN Model: A Predictive Mortality Score for Antisynthetase Syndrome

Chen Zong, G C Wang, Yiran Chen, Yingfang Zhang, Qinglin Peng, Xiaoming Shu, Yinli Zhang, Qi Pan, L Zhu, S Li, H Cai, Shiyu Wu, Xinxin Zhang, Xin Lu, Chao Sun
article en

Abstract

Objectives To develop and validate a prognostic model for predicting all‐cause mortality in patients with antisynthetase syndrome (ASyS). Methods This retrospective study included 1194 patients with ASyS from three independent institutions in China. The Cox proportional hazards (CPH) method and random survival forest (RSF) algorithm were used for developing a mortality risk prediction model in derivation cohort (n=763). The optimal model was simplified into a scoring system and validated in an external cohort of 431 patients from two institutions. Results The model constructed using the CPH method in the training cohort incorporated five prognostic factors: age at onset, lactate dehydrogenase, albumin, respiratory failure, and neutrophil‐to‐lymphocyte ratio. This model demonstrated better performance than the model developed using the RSF algorithm in the internal validation cohort and was subsequently transformed into a simplified scoring system, termed the ALARN score. The ALARN model achieved time‐dependent areas under the receiver operating characteristic curves of 0.914 (95% CI: 0.866–0.955), 0.867 (95% CI: 0.829–0.906), 0.839 (95% CI: 0.789–0.885), and 0.854 (95% CI: 0.789–0.909) for predicting 1‐, 3‐, 5‐, and 10‐year mortality, respectively. Moreover, patients were effectively stratified into low‐, intermediate‐, and high‐risk groups (ALARN scores of 0–2, 3–5, and 6–8, respectively) with significantly different survival outcomes (log‐rank p&lt;0.0001). Internal and external validation confirmed the robustness of the ALARN score in predicting overall mortality in ASyS. Conclusions The ALARN score incorporates five readily available clinical parameters and provides a simple, practical tool for predicting mortality risk in patients with ASyS.

Arthritis & Rheumatology
Chinese Academy of Medical Sciences & Peking Union Medical College (CN), China-Japan Friendship Hospital (CN), Zhengzhou University (CN), Nanjing Drum Tower Hospital (CN)
Good health and well-being
Openalex Percentile: Top 7%
Inflammatory Myopathies and Dermatomyositis
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