Abstract Anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive amyopathic dermatomyositis is a rare autoimmune condition characterized by minimal or absent muscle involvement and a strong association with rapidly progressive interstitial lung disease (RP-ILD), a clinical entity with a fulminant course and high mortality. We report the case of a 35-year-old Filipino female who presented with a three-week history of progressive dyspnea, body malaise, and intermittent fever, initially treated as severe community-acquired pneumonia and acute respiratory distress syndrome (ARDS) without improvement despite broad-spectrum antibiotics. Work up revealed hypoxemia, diffuse bilateral pulmonary infiltrates, and cutaneous findings including hyperpigmented plaques over the elbows and periungual erythema. Despite proning and empiric antimicrobials, worsening hypoxemia necessitated endotracheal intubation and venovenous extracorporeal membrane oxygenation (VV-ECMO). A myositis-specific antibody panel was done and came out positive for anti-MDA5 antibody, confirming the diagnosis of anti-MDA5-associated RP-ILD. This case exemplifies the aggressive nature of anti-MDA5-associated RP-ILD, which can initially mimic severe infection or ARDS, leading to delayed diagnosis and treatment. Optimal management requires early recognition and prompt initiation of intensive immunosuppression. VV-ECMO serves as an important bridge to recovery or transplantation in severe cases. This report highlights the importance of heightened clinical suspicion, multidisciplinary collaboration, and the urgent need for accessible advanced therapies for anti-MDA5 RP-ILD, particularly in resource-limited settings where transplant programs remain scarce. This abstract is funded by: None
Saqui et al. (Fri,) studied this question.