Frontiers in immunology · 2026
Clinical phenotype and prognostic determinants of spontaneous pneumomediastinum in anti-MDA5 antibody-positive dermatomyositis.
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Wang Xiaoying, Zhang Yusheng, Liu Lijun, Li Wei, Liu Xiaojun, Wang Yan
Аннотация
INTRODUCTION: To identify the clinical phenotype and prognostic determinants in patients with anti-MDA5 antibody-positive dermatomyositis (MDA5+DM) with spontaneous pneumomediastinum (SPM).
METHODS: We retrospectively analyzed 150 MDA5+DM patients (50 with SPM and 100 without). Clinical, laboratory, and therapeutic profiles were analyzed. Multivariable logistic regression was used to identify factors associated with survival in the SPM cohort.
RESULTS: The SPM group had a significantly higher 6-month mortality than the non-SPM group (54% vs. 16%, P<0.001). Patients with SPM were older and less frequently had arthralgia, and exhibited a hyper-inflammatory phenotype [elevated CRP, serum ferritin (SF), LDH, neutrophil-to-lymphocyte ratio (NLR), KL-6], severe lymphopenia, and higher infection rates. Prior calcineurin inhibitor (CNI) use was more common in the non-SPM group (85% vs. 62%, P = 0.001). Within the SPM group, non-survivors had shorter disease duration and higher inflammatory markers. Multivariable analysis identified longer pre-SPM disease duration (OR 0.479, 95% CI 0.266-0.863) and prior cyclophosphamide (CTX) therapy (OR 0.034, 95% CI 0.003-0.405) as factors independently associated with improved survival (both P<0.05).
CONCLUSION: Older MDA5+DM patients with a hyper-inflammatory phenotype and fewer arthralgias are at increased risk for SPM, whereas prior CNI use may be protective. In SPM patients, elevated inflammatory markers predict poor survival, while longer pre-SPM disease duration and prior CTX therapy are associated with improved survival, suggesting early aggressive immunosuppression may improve outcomes.
METHODS: We retrospectively analyzed 150 MDA5+DM patients (50 with SPM and 100 without). Clinical, laboratory, and therapeutic profiles were analyzed. Multivariable logistic regression was used to identify factors associated with survival in the SPM cohort.
RESULTS: The SPM group had a significantly higher 6-month mortality than the non-SPM group (54% vs. 16%, P<0.001). Patients with SPM were older and less frequently had arthralgia, and exhibited a hyper-inflammatory phenotype [elevated CRP, serum ferritin (SF), LDH, neutrophil-to-lymphocyte ratio (NLR), KL-6], severe lymphopenia, and higher infection rates. Prior calcineurin inhibitor (CNI) use was more common in the non-SPM group (85% vs. 62%, P = 0.001). Within the SPM group, non-survivors had shorter disease duration and higher inflammatory markers. Multivariable analysis identified longer pre-SPM disease duration (OR 0.479, 95% CI 0.266-0.863) and prior cyclophosphamide (CTX) therapy (OR 0.034, 95% CI 0.003-0.405) as factors independently associated with improved survival (both P<0.05).
CONCLUSION: Older MDA5+DM patients with a hyper-inflammatory phenotype and fewer arthralgias are at increased risk for SPM, whereas prior CNI use may be protective. In SPM patients, elevated inflammatory markers predict poor survival, while longer pre-SPM disease duration and prior CTX therapy are associated with improved survival, suggesting early aggressive immunosuppression may improve outcomes.