ArticleFrontiers in medicine2026
Clinical features and prognostic factors of anti-MDA5 antibody-positive dermatomyositis-associated interstitial lung disease with spontaneous mediastinal emphysema: a retrospective case series of nine patients.
Article in Frontiers in medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Objective: Spontaneous pneumomediastinum (SPM) is a life-threatening complication of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5 + DM) associated interstitial lung disease (ILD), particularly in patients with rapidly progressive ILD (RP-ILD), which carries an extremely high mortality rate. This study aimed to investigate the clinical characteristics, imaging features, and prognostic factors of MDA5 + DM-ILD patients with SPM to facilitate early detection and risk stratification of this high-risk population. Methods: The study retrospectively analyzed the clinical data of nine patients diagnosed with MDA5 + DM-ILD complicated by SPM at the center between June 2022 and February 2025. Collected data included demographic characteristics, laboratory biomarkers [including serum ferritin and lactate dehydrogenase (LDH)], high-resolution computed tomography (HRCT) findings assessed through the validated MDA5-specific computed tomography (MDA5-CT) scoring system, treatment regimens, and clinical outcomes. Results: The cohort included 4 male and 5 female patients, with a median age of 62 years (range 42-69 years). All patients presented with RP-ILD and developed SPM during the disease course, with a median hospital stay of 10 days (range 4-31 days). The overall mortality rate was 87.5% (7/8, excluding 1 patient lost to follow-up). All non-survivors had high-risk MDA5-CT scores (>18 points), accompanied by significantly elevated serum ferritin [median 1936 ng/mL, interquartile range (IQR) 1,119-3,204 ng/mL] and LDH (median 433 U/L, IQR 327-646 U/L). The only surviving patient had a low-risk MDA5-CT score (≤18 points). Conclusion: Patients with MDA5 + DM-ILD-SPM carry an extremely poor prognosis and high short-term mortality. An MDA5-CT score >18 is a strong predictor of mortality, particularly within the first 6 months after onset, with the highest risk in the first 3 months. Combining elevated serum ferritin, increased LDH, and tracheal dilation (>18 mm) may aid in early identification of high-risk patients for timely intervention.
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