Lung transplant outcomes in myositis, systemic sclerosis and idiopathic pulmonary fibrosis: a multicentre retrospective analysis
Authors' 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.
Study context
- Population
- Interstitial lung disease (ILD) patients with idiopathic inflammatory myopathies (IIM), SSc or idiopathic pulmonary fibrosis (IPF) who underwent lung transplantation (2012–24) at two Canadian centres, Vancouver and Montréal
- Participants
- 22 / 32 / 64
- Exposure
- Lung transplant outcomes in IIM-ILD and SSc-ILD vs IPF
- IIMn = 22
- SScn = 32
- IPFn = 64reference
- Outcome
- Post-transplant survival
Methodological detailsmulticentre, two Canadian centres
- Multicentre
- Two Canadian centres
Main finding
As reported in the abstract
Median follow-up, IIM
2.8 years
Median follow-up, SSc
2.5 years
Median follow-up, IPF
3.6 years
Observational data: associations, not effects.
Further findings
IIM characteristics
- Clinically amyopathic at presentation
- 41%
- Anti-MDA5 DM, all with RP-ILD
- 45%
- Anti-synthetase syndrome
- 32%
- Overlap myositis
- 14%
- Other DM
- 9%
SSc characteristics
- Pulmonary hypertension
- 88%
- Severe pulmonary hypertension (PH)
- 31%
- Oesophageal dysmotility
- 78%
Pre- and post-transplant course
- IIM required more frequent pre-transplant intensive care unit (ICU) admission and emergency transplantation
- IIM had longer post-transplant ICU/hospital stays
Long-term survival in IIM and SSc subgroups
- No significant differences in IIM (by transplant urgency, extracorporeal membrane oxygenation (ECMO) support, amyopathy) or SSc (by severe PH, oesophageal dysmotility, transplant urgency)
Original publication
Lung transplant outcomes in myositis, systemic sclerosis and idiopathic pulmonary fibrosis: a multicentre retrospective analysis. Rheumatology (Oxford). Published 15 April 2026. doi:10.
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