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Published on: June 16, 2020

Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis

Benoit Le Goff1, Patrick Chérin, Alain Cantagrel

  • 1Le Mans General Hospital, Le Mans, France.

Arthritis and Rheumatism
|January 1, 2009
PubMed
Abstract

Insights

Spontaneous pneumomediastinum is a rare complication in dermatomyositis (DM) and polymyositis (PM). Absence of muscle weakness and severe lung issues predict poor survival, but treatment can lead to recovery.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Internal Medicine

Background:

  • Spontaneous pneumomediastinum is a rare but serious complication of inflammatory myopathies, including dermatomyositis (DM) and polymyositis (PM).
  • Understanding the characteristics and prognostic factors of pneumomediastinum in DM/PM is crucial for patient management.

Purpose of the Study:

  • To characterize spontaneous pneumomediastinum in patients with DM and PM.
  • To identify factors associated with poor survival in this patient cohort.

Main Methods:

  • Retrospective analysis of a multicenter series of DM/PM cases with pneumomediastinum.
  • Inclusion of published cases for a larger investigation of survival factors.

Main Results:

  • Eleven DM/PM cases with interstitial lung disease and pneumomediastinum were identified.
  • Clinically amyopathic DM was noted in 5 of 9 DM patients.
  • Poor survival was linked to absence of muscle weakness, low vital capacity, and low carbon monoxide diffusion capacity.

Conclusions:

  • Pneumomediastinum can precede or reveal DM with minimal muscle involvement.
  • Absence of muscle weakness and severe pulmonary disease indicate a poorer prognosis.
  • Corticosteroids and immunosuppressive therapy can lead to complete recovery in many cases.

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