Prognostic factors for long-term survival in anti-MDA5-positive dermatomyositis: a 10-year cohort study.
4 affiliations
- 1Department of Rheumatology and Immunology, The Second People's Hospital of Changzhou, The Third Affiliated Hospital of Nanjing Medical University, Changzhou, China.
- 2Department of Rheumatology & Immunology, Arthritis Clinical Diagnosis and Treatment Centre, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
- 3Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital, Nanjing University Medical School, Nanjing, China.
- 4Department of Respiratory and Critical Care Medicine, The Affiliated Drum Tower Hospital, Nanjing University Medical School, Nanjing, China.
Abstract
Background
Anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5+ DM) is a severe subtype frequently complicated by rapidly progressive interstitial lung disease (RP-ILD), leading to high early mortality. However, factors associated with long-term survival beyond 10 years remain largely uncharacterized. This exploratory analysis aimed to identify baseline factors associated with long-term all-cause mortality in a 10-year follow-up cohort using LASSO-Cox regression.Methods
This retrospective longitudinal study enrolled 84 inpatients diagnosed with anti-MDA5+ DM between 2010 and June 2014; surviving patients had more than 10 years of potential follow-up until March 2026. The primary endpoint was all-cause mortality. All candidate covariates, including clinical features, laboratory parameters, HRCT score, and initial treatment variables, were entered simultaneously into LASSO-penalized Cox regression with 10-fold cross-validation, and retained variables were directly entered into a multivariable Cox model. The proportional hazards assumption was tested, and bootstrap resampling was performed to assess selection stability. Survival was illustrated using Kaplan-Meier curves with log-rank tests.Results
Among the 84 patients (27 males and 57 females), 41 (48.8%) were alive at the final follow-up and 43 (51.2%) had died. As a descriptive observation, 25 surviving patients (29.8% of the total cohort) had maintained sustained drug-free remission for over 2 years. Four baseline factors were independently associated with long-term mortality. Older age (HR = 1.05 per 1-year increase, P < 0.001), fever on admission (HR = 2.08, P = 0.036), and higher HRCT score (HR = 1.94 per 1-point increase, P < 0.001) were associated with higher mortality, whereas concurrent arthritis was associated with lower mortality (HR = 0.45, P = 0.022). Male sex showed a trend toward higher mortality that was not statistically significant after adjustment (P = 0.086).Conclusion
Older age, admission fever, higher HRCT score, and absence of arthritis - all assessable at admission - were independently associated with all-cause mortality over long-term follow-up in the full anti-MDA5+ DM cohort; landmark analysis showed only arthritis and HRCT score remained associated with mortality after year 1. Given the limited sample size and lack of external validation, these exploratory findings are hypothesis-generating and require confirmation in independent multicenter cohorts.
Related
- Ommaya Reservoir-Based Cerebrospinal Fluid Chemotherapy for Leptomeningeal Metastasis in the Era of Targeted Therapy: Safety, Survival, and Exploratory Prognostic Factors from a Single-Center Cohort
- Smoking status and survival by cancer type and treatment intent in a population-based cohort with adjustment for major prognostic factors.
- Survival Patterns and Prognostic Factors Among Children Hospitalized with Severe Malaria in Mbujimayi, Democratic Republic of the Congo: A Multicenter Cohort Study.
- Survival Outcomes and Prognostic Factors in Elderly Patients with Hodgkin Lymphoma: A Retrospective Cohort Study
- Treatment-Free Interval as a Prognostic Factor for Survival Outcomes in Patients with Advanced or Recurrent Endometrial Cancer Treated with Pembrolizumab Plus Lenvatinib: A Multi-Institutional Real-World Cohort Study
BibTeX
@article{wang2026prognostic,
title = {{Prognostic factors for long-term survival in anti-MDA5-positive dermatomyositis: a 10-year cohort study.}},
author = {Yan Wang and Xueliang Zhang and Xiaolei Ma and Xin Zhang and Sheng-Ming Dai and Yin Liu and Shengnan Zhao and Zhiyong Chen},
journal = {Frontiers in medicine},
year = {2026},
month = oct,
doi = {10.3389/fmed.2026.1908926},
url = {https://europepmc.org/article/MED/42923589},
note = {CC BY},
}
RIS
TY - JOUR
TI - Prognostic factors for long-term survival in anti-MDA5-positive dermatomyositis: a 10-year cohort study.
AU - Yan Wang
AU - Xueliang Zhang
AU - Xiaolei Ma
AU - Xin Zhang
AU - Sheng-Ming Dai
AU - Yin Liu
AU - Shengnan Zhao
AU - Zhiyong Chen
PY - 2026
DA - 2026/10/08
JO - Frontiers in medicine
DO - 10.3389/fmed.2026.1908926
UR - https://europepmc.org/article/MED/42923589
AB - Background Anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5+ DM) is a severe subtype frequently complicated by rapidly progressive interstitial lung disease (RP-ILD), leading to high early mortality. However, factors associated with long-term survival beyond 10 years remain largely uncharacterized. This exploratory analysis aimed to identify baseline factors associated with long-term all-cause mortality in a 10-year follow-up cohort using LASSO-Cox regression.Methods This retrospective longitudinal study enrolled 84 inpatients diagnosed with anti-MDA5+ DM between 2010 and June 2014; surviving patients had more than 10 years of potential follow-up until March 2026. The primary endpoint was all-cause mortality. All candidate covariates, including clinical features, laboratory parameters, HRCT score, and initial treatment variables, were entered simultaneously into LASSO-penalized Cox regression with 10-fold cross-validation, and retained variables were directly entered into a multivariable Cox model. The proportional hazards assumption was tested, and bootstrap resampling was performed to assess selection stability. Survival was illustrated using Kaplan-Meier curves with log-rank tests.Results Among the 84 patients (27 males and 57 females), 41 (48.8%) were alive at the final follow-up and 43 (51.2%) had died. As a descriptive observation, 25 surviving patients (29.8% of the total cohort) had maintained sustained drug-free remission for over 2 years. Four baseline factors were independently associated with long-term mortality. Older age (HR = 1.05 per 1-year increase, P < 0.001), fever on admission (HR = 2.08, P = 0.036), and higher HRCT score (HR = 1.94 per 1-point increase, P < 0.001) were associated with higher mortality, whereas concurrent arthritis was associated with lower mortality (HR = 0.45, P = 0.022). Male sex showed a trend toward higher mortality that was not statistically significant after adjustment (P = 0.086).Conclusion Older age, admission fever, higher HRCT score, and absence of arthritis - all assessable at admission - were independently associated with all-cause mortality over long-term follow-up in the full anti-MDA5+ DM cohort; landmark analysis showed only arthritis and HRCT score remained associated with mortality after year 1. Given the limited sample size and lack of external validation, these exploratory findings are hypothesis-generating and require confirmation in independent multicenter cohorts.
SN - 2296-858X
ER -