Extracorporeal membrane oxygenation for idiopathic inflammatory myopathy-associated interstitial lung disease: a...

Idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD), particularly its rapidly progressive form (RP-ILD), carries a grave prognosis, with refractory respiratory failure being a leading cause of death. For patients with IIM-ILD who progress to refractory respiratory failure, extracorporeal membrane oxygenation (ECMO) can bridge patients to recovery or transplantation. This review synthesizes existing literature on the role of ECMO in managing IIM-ILD with severe respiratory failure.

PubMed was systematically searched from its inception to August 31, 2025, for studies focusing on the use of ECMO in the management of IIM-ILD. Eligible studies were full-text articles published in English in peer-reviewed journals; studies were excluded if they involved patients under 18 years of age or if individual patient data could not be extracted.

Current evidence, primarily from case reports and case series, indicates that for IIM-ILD patients with severe respiratory failure, the primary value of ECMO lies in serving as a bridge to lung transplantation, with a markedly higher survival rate compared to its use solely as a bridge to recovery. However, this result may be influenced by publication bias, survivor bias, and center-level variations in clinical practices. The outcomes of patients with IIM-ILD are highly heterogeneous, influenced by myositis-specific antibody subtypes, fluctuations in antibody titers, and the presence of modifiable triggering factors. The review underscores the critical challenges of early diagnosis-often delayed due to atypical presentations-and the imperative for both early, aggressive, combination immunosuppressive therapy and evaluation for lung transplantation. The future role of ECMO in IIM-ILD remains a critical question mark, depending less on technology and more on refining patient selection, optimizing immunosuppressive regimens, integrating ECMO into proactive treatment pathways, and establishing ethical frameworks to improve meaningful outcomes for this complex population.

In IIM-ILD with refractory respiratory failure, ECMO primarily bridges selected patients to transplantation rather than recovery, though evidence is limited and biased. Given extreme disease heterogeneity, success hinges on patient selection and integrating ECMO into a proactive strategy combining early immunosuppression with timely transplant evaluation. Collaborative research is urgently needed.