Extracorporeal Membrane Oxygenation for Acute Lung Injury in Idiopathic Inflammatory Myopathies – a Potential Lifesaving Intervention

boyang zheng¹, Ellen Eline², Lilian Xu³, Kun Huang⁴, Greet Hermans⁵, michael Perch⁶, gordan Samoukovic¹, ellen De Langhe⁵, Maryam Dastmalchi⁷, Lisa Christopher-Stine², Louise Diederichsen⁸ and Valerie Leclair¹, ¹McGill University, Montréal, QC, Canada, ²Johns Hopkins University, Baltimore, MD, ³Johns Hopkins University School of Medicine, Baltimore, MD, ⁴University of British Columbia, Vancouver, BC, Canada, ⁵University Hospitals Leuven, Leuven, Belgium, ⁶Righospitalet, University of Copenhagen, Copenhagen, Denmark, ⁷Karolinska University Hospital, Stockholm, Sweden, ⁸Copenhagen University Hospital, Rigshospitalet, Dragør, Denmark

Meeting: ACR Convergence 2022

Keywords: dermatomyositis, interstitial lung disease, Myopathies

Session Information

Date: Saturday, November 12, 2022

Title: Muscle Biology, Myositis and Myopathies Poster I

Session Type: Poster Session A

Session Time: 1:00PM-3:00PM

Background/Purpose: Idiopathic inflammatory myopathies (IIM) can present with acute IIM-related lung injury and respiratory failure, leading to a high mortality risk in intensive care units (ICU). Extracorporeal membrane oxygenation (ECMO) is a last resort measure in patients with acute respiratory distress syndrome (ARDS) that can be lifesaving. We aimed to report outcomes of patients with IIM receiving ECMO to better inform clinicians on its use in these diseases.

Methods: Patients diagnosed with IIM from tertiary care centers in Belgium, Canada, Denmark, United States, and Sweden who underwent ECMO were reviewed to identify clinical characteristics and describe disease outcomes and hospitalization course. Clinical characteristics at admission and during ICU stay including ECMO complications and mortality causes were summarized for ICU survivors and those who died. Comorbidity burden was measured using the Charlson Comorbidity Index.

Results: The study included 25 patients (56% female, mean age at admission 47 ± 11 years, median disease duration 3 months [IQR 2, 10]) with dermatomyositis (64%), anti-synthetase syndrome (24%), polymyositis (8%) and overlap myositis (4%) (Table 1). IIM patients had low comorbidity scores and half of them (52%) received immunosuppression before their ICU admission. In the group of patients that survived, 62% were females compared to 44% in the non-survivor group and survivors were younger (44±11 vs 52±10). With regards to in-hospital treatment, 63% of ICU survivors were on >2 immunosuppressors compared to 88% in the non-survivor group. Among the 16 (64%) ICU survivors, 8 (32%) were bridged to transplant and 13 patients (52%) survived beyond 1 year after discharge (Figure 1). When comparing ICU survivors to non-survivors, those who survived were frequently found to have concomitant infections at ICU admission (75% vs 44%) and sepsis (75% vs 56%) (Table 2). Both groups had similar frequencies of ECMO-related complications (38% vs 44%).

Conclusion: ECMO was a lifesaving intervention in a majority of IIM patients presenting with acute IIM-related lung injury. ECMO allowed the necessary time for immunosuppression to take effect, and in some cases, was a bridge to lung transplant in patients who would have otherwise died without intervention.

Table 1. Clinical characteristics at hospital admission of IIM patients exposed to ECMO

Figure 1. Survival and cause of death in IIM patients exposed to ECMO

Table 2. ICU outcomes of IIM patients exposed to ECMO

Disclosures: b. zheng, None; E. Eline, None; L. Xu, None; K. Huang, AbbVie/Abbott, Eli Lilly, Janssen, Pfizer; G. Hermans, None; m. Perch, PulmonX, Takeda, Therakos, AMBU, Boeringer-Ingelheim; g. Samoukovic, None; e. De Langhe, None; M. Dastmalchi, None; L. Christopher-Stine, Janssen, Boehringer-Ingelheim, Mallinckroft, EMD-Serono, Allogene, ArgenX; L. Diederichsen, None; V. Leclair, None.

To cite this abstract in AMA style:

zheng b, Eline E, Xu L, Huang K, Hermans G, Perch m, Samoukovic g, De Langhe e, Dastmalchi M, Christopher-Stine L, Diederichsen L, Leclair V. Extracorporeal Membrane Oxygenation for Acute Lung Injury in Idiopathic Inflammatory Myopathies – a Potential Lifesaving Intervention [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/extracorporeal-membrane-oxygenation-for-acute-lung-injury-in-idiopathic-inflammatory-myopathies-a-potential-lifesaving-intervention/. Accessed .

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