Background/Purpose: Lung transplantation (LT) is gaining ground in managing advanced ILD in SSc patients. However, concerns remain among surgeons due to SSc’s complexity, multiorgan involvement (including oesophagus), and heightened risk of post-transplant complications. To date, outcomes focused on survival rates, lacking a comprehensive evaluation of how LT and immunosuppressive therapy affect SSc disease activity and progression. The aim of the study was to evaluate in a cohort of SSc patients, survival, complications, and post-LT outcomes regarding extra-pulmonary involvement, disease activity according to the EUSTAR activity index, and progression of lung scleroderma function

Methods: A retrospective analysis was conducted on SSc patients post-lung transplantation. Outcome variables (mRss, FVC, ulcers, EUSTAR activity, NVC) were evaluated at baseline, 6 months, 1 year, and 2 years. The study aimed to assess cutaneous involvement, lung function, ulcers, and disease activity in these patients

Results: We evaluated 11 patients, 9 with dcSSc and 2 lcSSc. M/F=8/3. Median disease duration: 10.33 yrs (IQR 9,42). Main reason for LT was ILD-related end-stage lung disease (11/11); 6/11 patients had concomitant PAH. NSIP pattern at HRTC scan was the most common one (9/11). Bilateral LT was performed in all patients but 1, who underwent unilateral procedure. Maintenance therapy with tacrolimus was the standard of care after LT and the mean dosage decreased from 4.31 mg at 6 months to 1.22 at 2 years. Perioperative complications included 4 acute rejects, with only 1 exitus. The survival at 1 and 2 years was of 100%, and 81,8% respectively. Mean value of FVC significantly increased from 42±10% at baseline, to 63.6±18% at 6 months, 69.8±22% at 1 year, and 76.6±6% at 2 years (p< 0.001 for all). Mean mRSS at baseline was 6.8 decreasing at 6 months (4.6), 1 yr (4.2) and 2yr (1.5) (p< 0.0001). 7 patients with active ulcers before transplantation experienced their resolutions after 6 months, with just one patient relapsing after 1 year, and another one after 2 years. In the same patients, the NVC features improved from late to active pattern. The EUSTAR activity index decreased progressively over time from 3.23 ±1.5 at baseline to 0,7 ±0.6 after 2 years (p< 0.0001)

Conclusion: To our knowledge this is the first study evaluating clinical outcomes and disease activity in SSc patients following LT. Previous studies have primarily focused on survival and post-transplant complications. The survival rate was 82% with no unexpected complications. Despite the underlying disease, we observed improvements in FVC, and disease activity scores consistently decreased after LT. These results were sustained throughout the follow-up period. Our findings confirm that LT is a viable therapeutic option for progressive, end-stage lung disease in SSc. Prolonged immunosuppressive therapy and improved tissue perfusion may contribute to the persistent reduction in disease activity.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/outcomes-in-patients-with-systemic-sclerosis-following-lung-transplantation-an-italian-multicentre-experience/