A severe anti MDA-5 antibodies associated amyopathic dermatomyositis.

BACKGROUND: Lung damage during amyopathic dermatomyositis (ADM) associated with auto antibodies anti MDA-5 is serious. We report a rare observation of a severe ADM associated to anti MDA-5 antibodies complicated with diffuse interstitial lung disease (ILD) rapidly extensive and fatal. OBSERVATION: We report the observation of a Tunisian adult who was admitted for ADM. The anti MDA-5 antibodies were detected. He developed an ILD rapidly extensive to fibrosis. Corticosteroids were unefficient and he deceded of pneumomediastin and refractory respiratory distress. CONCLUSION: This observation illustrates the severity of ADM with anti MDA-5 antibodies. Every clinician should detect any ILD to avoid the progression to fibrosis.