Spontaneous pneumomediastinum (SPM) is a rare complication of anti-melanoma differentiation-associated protein 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM). Herein, we report an elderly patient presented with cutaneous signs and SPM. Serial workup confirmed the anti-MDA5 positive CADM. Treatment initially included methylprednisolone, cyclosporine, and cyclophosphamide, but recurrence led to the use of rituximab and mycophenolate mofetil, ultimately resolving the pneumomediastinum and stabilisation of the lung functions. This underscores the importance of promptly recognising and managing pneumomediastinum in the context of anti-MDA5 positive CADM-associated rapid interstitial lung disease (RP-ILD).
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