Abstract Introduction Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) is a rare but devastating form of autoimmune lung disease, characterized by extensive alveolar damage, poor response to immunosuppression, and high mortality within a few months of diagnosis. Unlike other inflammatory ILDs, MDA5-positive CADM often follows an irreversible course despite aggressive therapy. Lung transplantation remains the only curative option once respiratory failure becomes refractory. Case Summary A previously healthy 67-year-old woman presented with a three-month history of progressive dyspnea and hypoxemia unresponsive to outpatient antibiotics. CT imaging revealed diffuse ground-glass opacities and consolidations consistent with acute respiratory distress syndrome (ARDS). She required intubation and lung-protective ventilation but rapidly deteriorated, necessitating veno-venous extracorporeal membrane oxygenation (VV-ECMO) for refractory hypoxemia. Autoimmune testing was positive for anti-MDA5 antibodies, establishing a diagnosis of anti-MDA5 CADM-associated RP-ILD. Despite early initiation of high-dose corticosteroids, rituximab, cyclophosphamide, calcineurin inhibitors, and plasmapheresis, the patient remained ECMO-dependent. She was transferred for lung transplant evaluation, but her course was complicated by empyema, and she ultimately died despite maximal therapy. Discussion MDA5-positive CADM-associated RP-ILD can cause rapidly progressive, irreversible lung injury even with optimized immunosuppression. Once ECMO support becomes necessary, survival depends primarily on timely lung transplantation rather than further medical escalation. ECMO should serve as a bridge to transplant—not a definitive therapy—given the high risk of infection and minimal potential for pulmonary recovery. Recent ELSO database meta-analyses indicate that delays of more than 10 days after ECMO initiation reduce survival rates to below 30%. Early multidisciplinary coordination among critical care, rheumatology, and transplant teams is essential to identify irreversible phenotypes and expedite transfer before systemic complications preclude candidacy. This abstract is funded by: None
Corpuz et al. (Fri,) studied this question.