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
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…
How strong is the evidence?
Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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