OP0288 OUTCOMES IN PATIENTS WITH SYSTEMIC SCLEROSIS FOLLOWING LUNG TRANSPLANTATION: AN ITALIAN MULTICENTRE EXPERIENCE
Authors: C. Iannone, M. R. Pellico, L. C. Morlacchi, V. Rossetti, M. Vicenzi, G. Trignani, P. Airo’, M. Saracco, A. Iagnocco, L. Beretta, A. Severino, E. Zaccara, F. Cacciapaglia, S. Stano, V. Codullo, F. Meloni, S. Cavalli, A. Minniti, P. Faggioli, F. Blasi, R. F. Caporali, N. Del Papa
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Lung transplantation (LT) shows promise for managing advanced interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. However, concerns remain among surgeons due to SSc multiorgan involvement, complexities related to esophageal dysfunction, and heightened post-transplant complications. Although literature has focused on survival rates, comprehensive studies about LT outcomes with particular regard to SSc activity and progression are still lacking. <h3>Objectives:</h3> To evaluate in SSc patients who underwent LT: 1) survival and complications after LT, 2) lung function, 3) progression of extra-pulmonary involvement, 4) disease activity evaluated by the EUSTAR disease activity index. <h3>Methods:</h3> We performed a retrospective analysis of SSc patients treated with LT, evaluating survival, cutaneous involvement by the modified Rodnan skin score (mRSS), lung function, digital ulcers, and EUSTAR disease activity at baseline, 6 months, 1 year, and 2 years post-LT. <h3>Results:</h3> We evaluated 13 SSc patients, 9 with diffuse cutaneous SSc (dcSSc) and 4 with limited cutaneous SSc (lcSSc). Patient demographics and clinical features are shown in Table 1. 12 patients underwent LT because of Interstitial lung disease (ILD)-related end-stage lung disease; one patient underwent LT for severe pulmonary arterial hypertension (PAH) complicated by veno-occlusive disease (PVOD). 77% had gastrointestinal involvement and 42% had acral ulcers, two of the most concerning extra-pulmonary manifestations in LT candidates. Bilateral LT was performed in all but one patient, who underwent unilateral LT. Maintenance immunosuppressive therapy was carried out with tacrolimus (mean dosage decreased from 4.45 mg at 6 months to 1.41 mg at 2 years). Perioperative complications included 4 acute rejections, with 1 death. Survival was 100% at 1 year and 92.3% at 2 years. Compared to 56.5±24% predicted at baseline, mean FVC significantly increased to 70.6±15% at 6 months, 78.8±21% at 1 year, and 78.2±15% at 2 years (p&lt;0.001 for all). Mean mRSS decreased from 5.6 at baseline to 3.8 at 6 months (p=0.04), 3.2 at 1 year (p=0.01), and 2.9 at 2 years (p&lt;0.01). Furthermore, EUSTAR activity index decreased from 1.09 at baseline to 0.36 at 2 years (p&lt;0.006). Six patients had active digital ulcers at baseline, and all healed by 6 months with only one patient relapsing at 12 months. <h3>Conclusion:</h3> Our study confirms that SSc patients and end-stage lung disease treated with LT have favorable outcomes. 92.3% of transplanted patients had 2-year survival without unexpected complications. We observed that after LT most of patients improved lung function, skin involvement, and decreased SSc activity. Additionally, LT was effective in decreasing disease activity and reducing vascular complications. The immunosuppression and improved tissue perfusion after transplantation might contribute to decrease SSc activity in transplanted patients. Overall, this study supports the viability of LT for end-stage lung disease in SSc. Furthermore, we firstly show that LT not only improves lung function, but also decreases overall disease activity and severity in SSc patients. <h3>REFERENCES:</h3> [1] Gelber AC. Lung transplantation for scleroderma: Strength in numbers. Joint Bone Spine. 2018 Jan;85(1):5–7. [2] 5Schachna L, Medsger TA, Dauber JH, Wigley FM, Braunstein NA, White B, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2006 Dec;54(12):3954–61. <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>