J Rheum Dis.  2014 Dec;21(6):317-321. 10.4078/jrd.2014.21.6.317.

A Case of Dermatomyositis Complicated with Pneumomediastinum Successfully treated with Cyclosporine A: a Case Report and Review of Literature

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Chonbuk National University Medical School and Research Institute of Clinical Medicine of Chonbuk National University Hospital-Chonbuk National University, Jeonju, Korea. ywhim@jbnu.ac.kr

Abstract

Dermatomyositis (DM) is a systemic inflammatory disease affecting skeletal muscles and other organs. Spontaneous pneumomediastinum (PnM) has been previously reported as a rare complication of DM and it is known to occur more frequently in patients with interstitial lung disease (ILD). Here we report on a case of a 52-year-old woman with DM who developed spontaneous PnM, which was treated successfully with high-dose steroid pulse therapy and cyclosporine A (CsA). This case suggests that CsA can be an effective therapeutic agent in DM refractory to glucocorticoid therapy, with ILD or pulmonary fibrosis accompanied by DM. CsA should be considered as an initial immunosuppressive agent for patients with PnM in DM.

Keyword

Dermatomyositis; Pneumomediastinum; Interstitial lung disease; Cyclosporin

MeSH Terms

Cyclosporine*
Dermatomyositis*
Female
Humans
Lung Diseases, Interstitial
Mediastinal Emphysema*
Middle Aged
Muscle, Skeletal
Pulmonary Fibrosis
Cyclosporine

Figure

  • Figure 1. Periungual erythema (A) and Gottron's sign (B).

  • Figure 2. Spontaneous pneumomediastinum (A) with subcutaneous emphysema (C) and resolved state (B and D) after the treatment with high dose steroid and cyclosporine.


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