Article
Acta Reumatológica Portuguesa
Case-based Review
Case-based Review
Spontaneous pneumomediastinum, a rare manifestation of clinically amyopathic dermatomyositis
Abstract
Clinically amyopathic dermatomyositis (CADM) is a rare condition characterized by
dermatomyositis skin lesions without clinically apparent muscle involvement. Respiratory
involvement is common, occurring in about half of the cases. Spontaneous
pneumomediastinum (PnM) is a rare, and often fatal, complication of CADM. We report a
case of a 61-year-old female patient who was diagnosed with anti-melanoma differentiation-
associated gene 5 antibody-associated CADM and interstitial lung disease. She developed
an extensive spontaneous PnM with subcutaneous emphysema. The patient was treated
with a conservative approach which was, initially, successful in reducing the size of the PnM.
However, the patient died from an eventual nosocomial pneumonia requiring mechanical
ventilation. This case illustrates that improving the management of CADM associated PnM,
remains a major unmet need.
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Publication:
2022-08-03
Cite:
Margarida Lucas Rocha, Laura Gago, Alexandre Sepriano, Tiago Saldanha, Mourão AF, Manuela Costa, Sandra André, Jaime C. Branco. Spontaneous pneumomediastinum, a rare manifestation of clinically amyopathic dermatomyositis. ARP Rheumatology, Vol 1, nº3 2022:251-254
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