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Vol. 5. Issue 2.
Pages 76-79 (March - April 2009)
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Vol. 5. Issue 2.
Pages 76-79 (March - April 2009)
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Pneumomediastinum and diffuse alveolar pain. Severe interstitial pneumopathy due to dermatomyositis
Neumomediastino y daño alveolar difuso. Afección pulmonar severa por dermatomiositis
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Juan Ramón de Diosa,
Corresponding author
jrdedios@htxa.osakidetza.net

Corresponding author.
, Ana Julia López de Goikoetxeaa, Juan Carlos Vesgaa, Laura Tomásb, Vanesa Zorrillab, José Luis Lobob
a Department of Rheumatology, Hospital de Txagorritxu, Vitoria, Álava, Spain
b Department of Pneumology, Hospital de Txagorritxu, Vitoria, Álava, Spain
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Abstract

We have recently observed the case of a 36-year-old man with dermatomyositis of recent onset, who developed massive pneumomediastinum and subcutaneous emphysema at the onset of a progressive and severe pulmonary disease. Although there were no sign of parenchymal cysts, after the bronchoscopy it was possible to observe endobronchial necrotic injury, which was considered as the likely source of the air leak. He was treated with high dose of corticosteroids, cyclophosphamide and cyclosporin A that resulted in the disappearance of the pneumomediastinum and subcutaneous emphysema and the progressive improvement of both parenchymal lung disease and respiratory insufficiency, enabled us to progressively taper the dose of corticosteroids.

Keywords:
Dermatomyositis
Pneumomediastinum
Subcutaneous emphysema
Resumen

Presentamos el caso de un varón de 36 años con dermatomiositis de reciente comienzo, que desarrolló neumomediastino y enfisema subcutáneo masivo al inicio de una neumopatía intersticial progresiva y severa. En el momento del diagnóstico no había imágenes parenquimatosas quísticas evidentes; sin embargo, la broncoscopia permitió evidenciar una lesión endobronquial de aspecto necrótico que se consideró como probable origen de la fuga aérea. El paciente se trató con corticoides a dosis altas, ciclofosfamida y ciclosporina A, con resolución del neumomediastino y del enfisema subcutáneo. Con el tratamiento se observó una mejoría progresiva de la afección parenquimatosa pulmonar y de la insuficiencia respiratoria, lo que permitió la disminución progresiva de los corticoides.

Palabras clave:
Dermatomiositis
Neumomediastino
Enfisema subcutáneo
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Copyright © 2009. Sociedad Española de Reumatología and Colegio Mexicano de Reumatología
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