Neumomediastino espontáneo como complicación de enfermedad pulmonar intersticial, asociada con dermatomiositis clínicamente amiopática y anticuerpos anti-MDA5 positivos

Clinically amyopathic dermatomyositis comprises a special group of patients within the spectrum of dermatomyositis characterised 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 organising pneumonia who developed spontaneous pneumomediastinum during its clinical course.