Abstract

SIFUENTES-GIRALDO, Walter Alberto; GARCIA-VILLANUEVA, María Jesús  and  GOROSPE, Luis. Spontaneous pneumomediastinum complicating interstitial lung disease, associated with clinically amyopathic dermatomyositis and positive anti-MDA5 antibodies. Rev.Colomb.Reumatol. [online]. 2017, vol.24, n.4, pp.259-264. ISSN 0121-8123.

Clinically amyopathic dermatomyositis comprises a special group of patients within the spectrum of dermatomyositis characterized by the presence of typical skin lesions, minimal or absent muscle involvement, and increased risk of interstitial lung disease. The antibodies directed against the protein encoded by melanoma differentiation-associated gene 5 (MDA5) are present in a significant proportion of patients with clinically amyopathic dermatomyositis, who develop rapidly progressive interstitial lung disease, with high mortality and frequently complicated by the onset of spontaneous pneumomediastinum. A case is presented of an African patient with anti-MDA5 positive clinically amyopathic dermatomyositis and interstitial lung disease with tomography pattern of organizing pneumonia who developed spontaneous pneumomediastinum during its clinical course.

Keywords : Anti-MDA5 antibodies; Clinically amyopathic dermatomyositis; Amyopathic dermatomyositis; Dermatomyositis; Interstitial lung disease; Spontaneous pneumomediastinum.

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