Anti-melanoma Differentiation-Associated Protein 5 (MDA5)-Positive Dermatomyositis With Rapidly Progressive Interstitial Lung Disease (ILD): A Rare and Lethal Entity to Recognize Early

Abstract

Anti-melanoma differentiation-associated protein 5 (anti-MDA5) clinically linked amyopathic dermatomyositis (CADM) is a rare autoimmune condition strongly linked to rapidly progressive interstitial lung disease (RP-ILD), a life-threatening complication. We present a 63-year-old female patient with anti-MDA5-positive CADM, who developed RP-ILD with an imaging pattern consistent with organizing pneumonia. She presented with Gottron's papules, periungual erythema, progressive dyspnea, and anorexia. Despite timely initiation of combination immunosuppressive therapy with corticosteroids, rituximab, and mycophenolate, her disease progressed rapidly, complicated by infections and treatment intolerance, culminating in acute respiratory failure. This case underscores the aggressive nature of anti-MDA5-positive CADM with RP-ILD, highlighting the critical need for prompt recognition and comprehensive multidisciplinary care. Urgent research efforts are essential to develop and refine treatment strategies for this life-threatening disease.

Keywords: anti-mda5 amyopathic dermatomyositis; autoimmune myopathy; immunosuppressive therapy; infectious lung disease; organizing pneumonia; rapidly progressive interstitial lung disease.