AB1483 CLINICAL OUTCOMES OF EXTRACORPOREAL LIFE SUPPORT AND LUNG TRANSPLANTATION IN SEVERE ANTI-MELANOMA DIFFERENTIATION-ASSOCIATED GENE 5 (MDA5)-RAPIDLY PROGRESSIVE INTERSTITIAL LUNG DISEASE: A SYSTEMATIC REVIEW

Background Anti-melanoma-differentiation-associated gene-5 antibody (MDA5) associated dermatomyositis is associated with rapidly progressive interstitial lung disease (RP-ILD), high mortality, and progression despite aggressive immunosuppression. Extra corporeal membrane oxygenation (ECMO), as bridge to recovery or bridge to transplantation and lung transplantation offer potentially lifesaving therapy for MDA5 RP-ILD but their roles are unclear, and its use in practice is often debated[1-4]. Objectives We systematically reviewed the clinical outcomes of patients with MDA5 RP-ILD receiving ECMO and/or lung transplantation. Methods Ovid Medline, Embase and CINAHL were searched (inception to Jan 2023) using keywords to identify studies of ECMO and lung transplantation in MDA5 RP-ILD. We included English language studies in adults with MDA5 RP-ILD requiring ECMO and/or lung transplantation. Review was prospectively registered on PROSPERO (CRD42022355450). Results The sixteen identified manuscripts reported outcomes for 33 patients with serological evidence of MDA5 (14 case reports, 2 case series). Most presented with respiratory symptoms, classic dermatomyositis cutaneous features, were amyopathic and had pulmonary radiological ground glass opacifications. Time to clinical diagnosis was usually less than 3 months from symptom onset, with rapid clinical deterioration from severe symptoms requiring hospitalisation to institution of mechanical ventilatory support within 2 weeks. Combination multi-modal aggressive induction immunosuppressants (corticosteroid, cyclophosphamide, tacrolimus, tofacitinib, rituximab and/or immunoglobulin) was used in 15/16. ECMO was used in 32 patients (Figure1): as a bridge to recovery in 16, and a bridge to transplantation in 16. ECMO used as a bridge to recovery had poor outcomes (15/16 deceased, 94%) mainly due to withdrawal of care. ECMO used as bridge to transplantation had an 87.5% survival rate (14/16), with MDA5 disease-free remission for up to 12 years using standard induction transplant immunosuppressants. Conclusion In refractory MDA5 RP-ILD, early consideration of lung transplantation is essential. The findings do not support ECMO as bridge to recovery given its high mortality rate. ECMO as bridge to transplantation provided excellent patient survival and should be considered as potentially lifesaving. References [1]Mehta P, Hoyles RK, Gunawardena H, Gooptu B, Chaudhuri N, Heightmann MJ, et al. Rheumatologists have an important role in the management of interstitial lung disease (ILD): a cross-speciality, multi-centre, UK perspective. Rheumatology. 2022;61(5):1748-1751. [2]Romero-Bueno F, Diaz Del Campo P, Trallero-Araguás E, Ruiz-Rodríguez JC, Castellvi I, Rodriguez-Nieto MJ, et al. Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum. 2020;50(4):776-790. [3]Rubin J, Black KE, Hallowell RW, Witkin AS, Lydston M, Shelton K, et al. Veno-Venous Extracorporeal Membrane Oxygenation for Myositis-Associated Rapidly Progressive Interstitial Lung Disease. Chest. 2021;160(6):2163-2167. [4]Bay P, de Chambrun MP, Roux A, Bunel V, Combes A, Biet DI, et al. Extracorporeal Life Support Allows Lung Transplant in anti-MDA5+ Rapidly Progressive-Interstitial Lung Disease. Eur Respir J. 2022:2102968. Acknowledgements: NIL. Disclosure of Interests Yuan Lim: None declared, Jessica Fairley: None declared, Yuanyuan Wang: None declared, Jyotika Prasad: None declared, Anita Wluka: None declared, Rohit Aggarwal Consultant of: 1.Mallinckrodt: Consultant 2.Octapharma: Consultant 3.CSL Behring: Consultant 4.Bristol Myers-Squibb: Consultant 5.EMD Serono: Consultant 6.Kezar: Consultant 7.Pfizer: Consultant 8.AstraZeneca: Consultant 9.Alexion: Consultant 10.Argenx: Consultant 11.Boehringer Ingelheim (BI): Consultant 12.Corbus: Consultant 13.Janssen: Consultant 14.Kyverna: Consultant 15.Roivant: Consultant 16.Merck: Consultant 17.Galapagos: Consultant 18.Actigraph: Consultant 19.Abbvie: Consultant 20.Scipher: Consultant 21.Horizontal Therapeutics: Consultant 22.Teva: Consultant 23.Biogen: Consultant 24.Beigene: Consultant 25.ANI Pharmaceutical: Consultant 26.Nuvig: Consultant 27.Capella: Consultant 28.CabalettaBio: Consultant, Grant/research support from: 1.Mallinckrodt 2.Pfizer 3.Bristol Myers-Squibb 4.Q32 5.EMD Serono 6.Janssen 7.Boehringer Ingelheim (BI): , Flavia Cicuttini: None declared