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

Abstract

To describe clinical characteristics and post-lung transplant outcomes of patients with idiopathic inflammatory myopathies (IIM), SSc and idiopathic pulmonary fibrosis (IPF). We retrospectively analysed interstitial lung disease (ILD) patients with IIM (n = 22), SSc (n = 32) and IPF (n = 64) who underwent lung transplantation (2012-24) at two Canadian centres, Vancouver and Montréal. Among IIM patients, 41% were clinically amyopathic at presentation, and 45% had anti-melanoma differentiation-associated protein 5 (anti-MDA5) 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 oesophageal dysmotility. IIM patients required more frequent pre-transplant intensive care unit (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, oesophageal dysmotility and transplant urgency) showed no significant differences in long-term survival. No autoimmune disease recurrence was observed. 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.