ArticleRheumatology (Oxford, England)2026
Lung transplant outcomes in myositis, systemic sclerosis and idiopathic pulmonary fibrosis: a multicentre retrospective analysis.
Article in Rheumatology (Oxford, England), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectivesTo describe clinical characteristics and post-lung transplant outcomes of patients with idiopathic inflammatory myopathies (IIM), SSc and idiopathic pulmonary fibrosis (IPF).
methodsWe 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.
resultsAmong 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.
conclusionDespite 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.
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