Successful treatment of rapidly progressive interstitial lung disease complicated by a refractory pneumomediastinum in a patient with anti‐MDA5 antibody‐positive dermatomyositis

Abstract

Abstract Introduction Anti‐melanoma differentiation‐associated gene 5‐positive dermatomyositis (anti‐MDA5+ DM) is a distinct subtype of DM, which is characterized by typical cutaneous features, minimal or no muscle involvement and notable interstitial lung disease, which typically progresses rapidly and has a high mortality. Spontaneous pneumomediastinum (PNM), a relatively unusual but serious complication of anti‐MDA5+ DM, further increases mortality. Currently, there is no generally accepted treatment regimen for anti‐MDA5+ DM‐associated PNM. Case Description A 53‐year‐old man with anti‐MDA5+ DM presented with rapidly progressive interstitial lung disease that progressed to diffuse subcutaneous emphysema and PNM despite aggressive immunosuppressive therapies. He responded well to combined anti‐infection treatments, moderate immunotherapy, and continuous oxygen therapy. Conclusion Comprehensive screening for potential infections, as well as close monitoring of the patient's immune status is essential for individualizing treatment and maximizing prognosis.

Keywords

• anti‐melanoma differentiation‐associated protein 5 antibody
• dermatomyositis
• interstitial lung diseases
• pneumomediastinum