Spontaneous pneumomediastinum in a dermatomyositis patient with anti-melanoma differentiation-associated gene-5 antibody and interstitial lung disease despite an initial response to immunosuppressant

Int J Rheum Dis. 2019 Mar;22(3):521-524. doi: 10.1111/1756-185X.13112. Epub 2017 Jun 4.

Abstract

We report a 24-year-old man with anti-melanoma differentiation-associated gene-5 (MDA5) antibody-positive dermatomyositis (DM) and interstitial lung disease (ILD) who developed spontaneous pneumomediastinum. By comparing serial thoracic high-resolution computed tomography scans, we demonstrated the distinct time course showing a paradoxical occurrence of pneumomediastinum despite a radiological improvement of ILD. Our case shows that pneumomediastinum in DM can occur regardless of associated ILD and it is a serious complication that should be considered in DM patients presenting with pulmonary manifestations. Cutaneous vasculopathy may be associated with pneumomediastinum and could potentially be a useful indicator of future disease.

Keywords: MDA5; dermatomyositis; interstitial lung disease; pneumomediastinum.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Dermatomyositis / complications*
  • Dermatomyositis / diagnosis
  • Dermatomyositis / drug therapy
  • Dermatomyositis / immunology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Interferon-Induced Helicase, IFIH1 / immunology*
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / drug therapy
  • Lung Diseases, Interstitial / etiology*
  • Lung Diseases, Interstitial / immunology
  • Male
  • Mediastinal Emphysema / diagnostic imaging
  • Mediastinal Emphysema / etiology*
  • Time Factors
  • Tomography, X-Ray Computed
  • Young Adult

Substances

  • Autoantibodies
  • Immunosuppressive Agents
  • IFIH1 protein, human
  • Interferon-Induced Helicase, IFIH1