A48-11 From Rapid Decline to Recovery: A Case of Early Identification and Expedited Lung Transplantation Evaluation in Anti-melanoma Differentiation-Associated Gene 5 Dermatomyositis With Rapidly Progressive Interstitial Lung Disease (Anti-MDA5 DM RP-ILD)

Introduction Anti-melanoma differentiation-associated gene 5 (anti-MDA5) dermatomyositis (DM) with rapidly progressive interstitial lung disease (RP-ILD) is an aggressive condition, with mortality rates approaching 80%. Upfront aggressive immunosuppression with high-dose glucocorticoids and cyclophosphamide forms the treatment cornerstone, however evidence supporting a specific regimen is limited. Lung transplantation, though controversial due to multisystem involvement and potential disease recurrence, is a lifesaving option when medical therapy fails. This case highlights the importance of early identification of anti-MDA5 DM RP-ILD and prompt assessment for lung transplantation given the rapid respiratory decline and high mortality rates despite intensive immunosuppression. Case Description A 38-year-old Chinese female presented to the hospital with a 3-month history of cough, dyspnea, fevers, arthralgias, and a rash after receiving empiric pneumonia treatment at two prior presentations. Her exam revealed hypoxia to 84% on room air requiring 2L of nasal cannula, tachypnea, diffuse crackles, and clubbing. She had a periorbital violaceous rash, an erythematous rash on her chest and upper back, and hyperkeratotic scaling on her hands with normal creatine kinase levels. Computed tomography chest imaging showed bilateral, lower lobe predominant peribronchovascular and subpleural opacities with interstitial thickening (Figure 1). By hospital day 3, she required high-flow-nasal-cannula (HFNC) 40L/70% FiO2 and immunosuppressive therapy with pulse-dose steroids, tofacitinib, and intravenous immunoglobulin (IVIG) for suspected amyopathic DM with RP-ILD (later confirmed as anti-MDA5+). She was then transferred to our center for expedited lung transplant evaluation. Given progressive respiratory failure, cyclophosphamide and plasmapheresis were added, however her oxygen requirement increased to HFNC 40L/100% by day 14. After multidisciplinary discussions, she was listed for transplant on day 18. She underwent bilateral lung transplant on day 25 and was extubated on post-op day 4. Post-transplant, her anti-MDA5 titers cleared, a favorable prognostic sign. Her heliotrope rash recurred, however this resolved with IVIG. At 3-month follow up, she remains off oxygen and without graft rejection, immune related adverse events or opportunistic infections. Discussion This case demonstrates the significance of early identification and successful navigation of the transplant pathway for a patient with anti-MDA5 DM RP-ILD and rapid respiratory decline despite aggressive immunosuppression and plasmapheresis. Evidence supporting early lung transplantation is limited, as it is viewed as rescue therapy. Patients suspected of having anti-MDA5 DM RP-ILD should be considered for immediate transfer to a transplant center as they may be candidates for a lifesaving transplantation. This aggressive approach is justified by the high mortality risk associated with this condition. This abstract is funded by: None