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Updated: Dec 21, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease: The FLAIR Model
Xinyue Lian1, Jing Zou2, Qiang Guo1
1Departments of Rheumatology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
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 to guide clinical treatment.
Research Question:
How to identify patients with ADM-ILD who are at high risk and to predict patient 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 patient data from January 2012 to December 2016. We used the index of prediction accuracy that uses 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 (<636 ng/mL, 0; ≥636 ng/mL, 2), lactate dehydrogenase (<355 U/L, 0; ≥355 U/L, 2), antimelanoma differentiation-associated gene 5 antibody (negative, 0; +, 2; ++, 3; +++, 4), high-resolution CT imaging score (<133, 0; ≥133, 3), and rapidly progressive ILD (RPILD) (non-RPILD, 0; RPILD, 2). We divided patients into three risk groups according to the FLAIR score: low, 0 to 4; medium, 5 to 9; and high, 10 to 13. In both discovery and validation cohorts, high-risk patients had significantly higher mortality rates than low- and medium-risk patients (P < .001).
Interpretation:
The FLAIR risk score model could help to predict survival in patients with ADM-ILD and to guide further clinical research on risk-based treatment.
Insights
A new FLAIR risk score helps predict survival in patients with amyopathic dermatomyositis-associated interstitial lung disease (ADM-ILD). This tool stratifies patients into low, medium, and high-risk groups, guiding clinical decisions for ADM-ILD.
Area of Science:
- Pulmonology
- Rheumatology
- Medical Diagnostics
Background:
- Amyopathic dermatomyositis-associated interstitial lung disease (ADM-ILD) carries a poor prognosis.
- There is a critical need for a validated mortality risk score to guide clinical management and treatment strategies for ADM-ILD patients.
Purpose of the Study:
- To develop and validate a risk score model for predicting survival in patients diagnosed with ADM-ILD.
- To identify independent prognostic risk factors for mortality in ADM-ILD.
Main Methods:
- A prospective inception cohort of 207 ADM-ILD patients was studied.
- A multivariable Cox proportional hazards model identified prognostic factors, leading to the creation of the FLAIR risk score.
- The model's accuracy was assessed using the Brier score and validated in an independent patient cohort.
Main Results:
- The FLAIR score incorporates ferritin, LDH, anti-MDA5 antibody levels, CT imaging score, and rapidly progressive ILD (RPILD).
- Patients were categorized into low (0-4), medium (5-9), and high (10-13) risk groups based on their FLAIR score.
- High-risk patients demonstrated significantly higher mortality rates compared to low- and medium-risk groups in both discovery and validation cohorts (P < .001).
Conclusions:
- The developed FLAIR risk score effectively predicts survival in patients with ADM-ILD.
- This risk stratification tool can guide clinical research and inform risk-based treatment strategies for ADM-ILD.
