Inflammatory myopathies

Spontaneous pneumomediastinum in anti-MDA5-positive dermatomyositis: Prevalence, risk factors, and prognosis

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

Nearly one-tenth of patients with anti-MDA5+ DM develop SPM. Both CMV and fungal infections are risk factors for SPM occurrence. The development of SPM does not worsen the prognosis of anti-MDA5+ DM patients, and the intensification of IST does harm to the SPM prognosis.

Study context

Population
Patients with idiopathic inflammatory myopathy (IIM), including anti-MDA5+ DM patients384 anti-MDA5+ DM
Participants
1,352 patients
Exposure
Anti-MDA5+ DM with spontaneous pneumomediastinum (SPM) vs without SPM
  • SPMn = 36
Outcome
Prevalence of SPM in anti-MDA5+ DM

Main finding

As reported in the abstract

SPM

9.4 %

36

Significantly higher than non-anti-MDA5+ DM and other IIM subtypes
P all <0.001

Observational data: associations, not effects.

Further findings

Time from anti-MDA5+ DM onset to SPM
5.5 · median · IQR 3.0–12.0 · months
Short-term and long-term survival · comparable survival
P = 0.236

Fever, dyspnea and pulmonary infection (incl. viral and fungal)

Significantly higher frequency in SPM vs non-SPM
P all < 0.05

Risk factors for SPM development

Cytomegalovirus (CMV) and fungal infections independent risk factors for SPM

Mortality in SPM (non-survivors vs survivors)

Non-survivors: lower peripheral lymphocyte count, higher LDH, more frequent intensification of IST
Elevated LDH and intensification of IST independent risk factors for increased mortality

Original publication

Spontaneous pneumomediastinum in anti-MDA5-positive dermatomyositis: Prevalence, risk factors, and prognosis. Semin Arthritis Rheum. Published 3 January 2024. doi:10.1016/j.semarthrit.2023.152352

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