Systemic sclerosis & Raynaud's
Pulmonary and extra-pulmonary effects of lung transplantation in an Italian cohort of patients with systemic sclerosis
Authors' conclusion
In SSc patients undergoing LuTx, an excellent 2-year survival was observed, without any disease-related adverse events.
Study context
- Population
- SSc patients with end-stage lung disease who underwent lung transplantation between 2010 and 2021, Italy (male/female 9/4; median age 48.7 years)
- Participants
- 13 patients
- Exposure
- Diffuse cutaneous scleroderma (dcSSc) vs limited cutaneous scleroderma (lcSSc)
- dcSScn = 9
- lcSScn = 4
- Outcome
- Patient survival and LuTx outcomes on systemic disease manifestations (mRSS, modified EUSTAR index 0-9, FVC)
- Follow-up
- 6, 12 and 24 months post-LuTx
Methodological detailssingle-group, Kaplan-Meier analysis
- Single-group
- Kaplan-Meier analysis
Main finding
As reported in the abstract
Survival rate
Survival rate at 2 years
92.3%
Survival rate at 5 years
76.9%
Observational data: associations, not effects.
Further findings
Forced vital capacity (FVC) (% predicted), baseline to 2 years
- FVC significantly increased from 56% of predicted at baseline to 78% at 2 years (P = 0.003)
Modified Rodnan skin score (mRSS) in dcSSc, decrease
- mRSS decreased from 7.4 ± 3.8 to 3.3 ± 2.5 in dcSSc (P = 0.02)
Modified EUSTAR index score, baseline to 2 years
- Modified EUSTAR index decreased from 2.54 ± 1.8 at baseline to 0.49 ± 0.5 at 2 years (P = 0.02)
Safety
- No unexpected adverse events observed
Original publication
Pulmonary and extra-pulmonary effects of lung transplantation in an Italian cohort of patients with systemic sclerosis. Rheumatology (Oxford). Published 19 September 2024. doi:10.
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