Extract
Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5) dermatomyositis (DM) is a rare subtype of idiopathic inflammatory myopathy, associated with severe interstitial lung disease (ILD) [1]. A subset of anti-MDA5 DM patients with rapidly progressive ILD (RP-ILD) have a very poor prognosis, with reported mortality rates reaching 80–84% [1, 2]. In this clinical setting, the use of extracorporeal life support (ECLS) is questionable, as reported in several studies that emphasise the futility of a bridge-to-recovery strategy [2, 3]. In this respect, emergency lung transplantation of previously unlisted patients on ECLS is under debate [4–6]. Yet, new therapeutic regimens, especially the early association of calcineurin inhibitors and cyclophosphamide [7], Janus-kinase inhibitors (JAKi) [8] or plasma exchange [9], have been recently associated with better outcomes of anti-MDA5 DM, fuelling hopes for a successful treatment of RP-ILD patients. Data on the outcome of anti-MDA5 RP-ILD requiring ECLS in the modern era are therefore urgently awaited.
Abstract
Anti-MDA5 RP-ILD has a poor prognosis and a high mortality rate. This study found that emergency lung transplantation in anti-MDA5 RP-ILD patients requiring ECLS has a favourable disease outcome, irrespective of prior treatment regimen.https://bit.ly/3gHKPt6
Acknowledgements
Hans Yssel and Bichat Lung Transplant Group.
Footnotes
Author contributions: P. Bay, M. Pineton de Chambrun and Y. Uzunhan designed the study, analysed the data and wrote the manuscript. Other authors included patients in the study.
Conflict of interest: None to disclose.
- ReceivedNovember 17, 2021.
- AcceptedFebruary 8, 2022.
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