JHLT open · 2026
The role of extracorporeal membrane oxygenation in the management of rapidly progressive interstitial lung disease due to anti-melanoma differentiation-associated gene 5 antibody dermatomyositis: A case series and brief literature review.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Jurica James M, Odish Mazen F, Lin Christine M, Owens Robert L, Yi Cassia, Parrett Michelle, Kabadi Alisha, LeBlanc Shannon, Roche Chelsea, Yung Gordon, Joshua Jisha, Choi Soo-In, Golts Eugene, Pollema Travis, Afshar Kamyar
Аннотация
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 63% mortality. 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, intended as a bridge to lung transplantation.
METHODS: A retrospective chart review was conducted on patients who were treated at our center between 2021 and 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 9 patients (5 females and 4 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, most commonly due to severe deconditioning, and died in the ICU. One patient was successfully bridged to bilateral lung transplant after 3 days on ECMO 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 (average time on ECMO 18 days), 46 (58.2%) died in the ICU (average time on ECMO 28 days), and only 4 (5%) were successfully bridged to recovery (average time on ECMO 44 days). Outcomes for 1 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 (∼5%, 4/79) for these patients; thus, lung transplant should be pursued expeditiously, particularly given the possible association between shorter time on ECMO and successful transplantation. 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.
METHODS: A retrospective chart review was conducted on patients who were treated at our center between 2021 and 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 9 patients (5 females and 4 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, most commonly due to severe deconditioning, and died in the ICU. One patient was successfully bridged to bilateral lung transplant after 3 days on ECMO 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 (average time on ECMO 18 days), 46 (58.2%) died in the ICU (average time on ECMO 28 days), and only 4 (5%) were successfully bridged to recovery (average time on ECMO 44 days). Outcomes for 1 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 (∼5%, 4/79) for these patients; thus, lung transplant should be pursued expeditiously, particularly given the possible association between shorter time on ECMO and successful transplantation. 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.