Lung transplant outcomes in myositis, systemic sclerosis and idiopathic pulmonary fibrosis: a multicenter retrospective analysis

Abstract Objectives To describe clinical characteristics and post-lung transplant outcomes of patients with idiopathic inflammatory myopathies (IIM),systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF). Methods We retrospectively analyzed interstitial lung disease (ILD) patients with IIM (n = 22), SSc (n = 32), and IPF (n = 64) who underwent lung transplantation (2012-2024) at two Canadian centers, Vancouver and Montréal. Results Among IIM patients, 41% was clinically amyopathic at presentation, and 45% had anti-MDA5 dermatomyositis (DM), all with rapid progressive (RP)-ILD, 32% anti-synthetase syndrome, 14% overlap myositis, and 9% other DM. In SSc, 88% had pulmonary hypertension (PH) (31% severe) and 78% had esophageal dysmotility. IIM patients required more frequent pre-transplant ICU admission and emergency transplantation. Post-transplant, IIM patients had longer ICU/hospital stays. There were no significant differences in 1-year survival, survival at last follow-up (median: 2.8 years for IIM, 2.5 years for SSc, and 3.6 years for IPF), incidence of chronic lung allograft dysfunction, or malignancy. Subgroup analyses of IIM (stratified by transplant urgency, extracorporeal membrane oxygenation (ECMO) support, and amyopathy) and SSc (stratified by severe PH, esophageal dysmotility, and transplant urgency) showed no significant differences in long-term survival. No autoimmune disease recurrence was observed. Conclusion Despite their underlying autoimmune diseases, post-transplant survival outcomes of selected IIM and SSc patients did not differ significantly from those with IPF. IIM patients with RP-ILD necessitating emergency transplantation and ECMO support exhibited survival similar to those without such complications. However, their more complex pre- and post-transplant courses emphasize the necessity for individualized lung transplant strategies and a multidisciplinary management approach.