Abstract Introduction Rapidly progressive interstitial lung disease (RP-ILD) from anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) dermatomyositis (DM) is a rare condition associated with up to 80% mortality within three months of diagnosis. Despite aggressive medical treatment, many patients require escalating levels of respiratory support. Extracorporeal membrane oxygenation (ECMO) has emerged as a rescue therapy for these patients and is generally intended as a bridge to lung transplantation. Methods A retrospective chart review was conducted on patients who were treated at our center between 2021-2025. Baseline characteristics, treatments, complications, and outcomes are described and presented herein. A literature review was performed to compare our outcomes for patients with anti-MDA5 DM placed on ECMO with those reported elsewhere. Results The study included nine patients (five females and four males) with RP-ILD due to anti-MDA5 DM. All patients were treated with high-dose corticosteroids and a variety of other immunosuppressive therapies. Eight patients were placed on veno-venous (V-V) ECMO, and one patient was placed on veno-arterialvenous (V-AV) ECMO. All patients were evaluated for lung transplant candidacy. Eight of nine patients were deemed ineligible for lung transplant due to deconditioning and infectious complications and died in the ICU. One patient was bridged to bilateral lung transplant and is currently 30 months post-transplant. A retrospective review of published studies found 79 patients with anti-MDA5 DM induced RP-ILD requiring ECMO support. Twenty-eight (35.4%) patients were bridged to transplant, 46 (58.2%) died in the ICU, and only four (5%) were successfully bridged to recovery. Outcomes for one patient were not reported. Conclusion RP-ILD due to anti-MDA5 DM is associated with a high rate of mortality despite current medical therapies. ECMO as a bridge to recovery is very unlikely for these patients, thus lung transplant should be pursued expeditiously. Immediate transfer to an ECMO and lung transplant center is crucial since immunosuppression decisions should be made in a multidisciplinary fashion (lung transplant, rheumatology, etc.) as these decisions can affect lung transplant candidacy. This abstract is funded by: None
Jurica et al. (Fri,) studied this question.