Extracorporeal life support allows lung transplant in anti-MDA5+ rapidly progressive-interstitial lung disease.

Abstract

Background: Anti-MDA5 dermatomyositis (DM) is a rare subtype of idiopathic inflammatory myopathy, associated with high incidence of rapidly-progressive interstitial lung disease (RP-ILD) with high mortality rate. The use of extracorporeal life support (ECLS) in these patients is questionable, as reported in several studies that emphasize the futility of a bridge-to-recovery strategy. Emergency lung transplantation of previously unlisted patients on ECLS is under debate.

Aims and objectives: To describe the outcome of anti-MDA5 RP-ILD patients requiring ECLS.

Methods: This French multicenter retrospective study conducted from 2013 to 2021 included all patients with anti-MDA5 RP-ILD requiring ECLS.

Results: 15 patients requiring ECLS were included (VV-ECMO n=13, VA-ECMO n=1, ECCO2R n=1). The female-to-male ratio was 4 and the age at ICU admission was 50 [32-67] years. The diagnosis of MDA5 DM was known at ECLS initiation for 8 patients. Five patients underwent lung transplantation after 8 [4-20] days on ECMO, none previously listed for a lung transplant. After a median follow-up of 25 [3-93] months, all transplanted patients were alive and no relapse of DM or ILD was noted. All other patients, not listed for lung transplantation, died after a median of 30 [4-52] days on ECMO.

Conclusions: The results provide crucial information for the management of anti-MDA5 RP-ILD. The bridge-to-recovery strategy in anti-MDA5 RP-ILD patients requiring ECLS despite specific prior treatment leads to undesirable results. In contrast, a bridge-to-emergency lung transplantation is not only feasible but also associated with a favorable outcome and appears therefore as the sole hope of survival.