Academic Journal
Pulmonary and extra-pulmonary effects of lung transplantation in an Italian cohort of patients with systemic sclerosis
| العنوان: | Pulmonary and extra-pulmonary effects of lung transplantation in an Italian cohort of patients with systemic sclerosis |
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| المؤلفون: | Iannone, Claudia, Pellico, Maria Rosa, Morlacchi, Letizia Corinna, Rossetti, Valeria, Vicenzi, Marco, Beretta, Lorenzo, Severino, Adriana, Airò, Paolo, Cacciapaglia, Fabio, Codullo, Veronica, Faggioli, Paola, Iagnocco, Annamaria, Meloni, Federica, Mercante, Lorena, Saracco, Marta, Stano, Stefano, Zaccara, Eleonora, Minniti, Antonina, Cavalli, Silvia, Trignani, Giorgia, Blasi, Francesco, Nosotti, Mario, Boffini, Massimo, Caporali, Roberto, Del Papa, Nicoletta |
| المساهمون: | C. Iannone, M.R. Pellico, L.C. Morlacchi, V. Rossetti, M. Vicenzi, L. Beretta, A. Severino, P. Airò, F. Cacciapaglia, V. Codullo, P. Faggioli, A. Iagnocco, F. Meloni, L. Mercante, M. Saracco, S. Stano, E. Zaccara, A. Minniti, S. Cavalli, G. Trignani, F. Blasi, M. Nosotti, M. Boffini, R. Caporali, N. Del Papa |
| بيانات النشر: | Oxford University Press |
| سنة النشر: | 2024 |
| المجموعة: | The University of Milan: Archivio Istituzionale della Ricerca (AIR) |
| مصطلحات موضوعية: | SSc, systemic sclerosi, lung, transplantation, disease activity, Eustar activity index, Settore MEDS-07/A - Malattie dell'apparato respiratorio, Settore MEDS-13/A - Chirurgia toracica |
| الوصف: | Objectives: Lung transplantation (LuTx) is a life-saving intervention for Systemic Sclerosis (SSc) patients with end-stage lung disease. The aim of this study was to evaluate patients' survival and LuTx outcomes on systemic disease manifestations. Methods: A retrospective evaluation was conducted on SSc patients who underwent LuTx between 2010 and 2021. Outcomes assessed at baseline, 6, 12, and 24 months post-LuTx included skin involvement by modified Rodnan skin score (mRSS), global disease activity using a modified EUSTAR index (0-9 scale). Lung function rescue was evaluated by forced vital capacity (FVC). Patient survival was assessed by Kaplan-Meier analysis. Results: 13 SSc patients were included, with a male/female ratio 9/4 and a median age of 48.7 years. Nine patients were affected by diffuse cutaneous scleroderma (dcSSc) and four by limited cutaneous scleroderma (lcSSc). FVC significantly increased from 56% of the predicted value at baseline to 78% at 2 years (p= 0.003). mRSS decreased from 7.4 ± 3.8-3.3 ± 2.5 in patients with dcSSc (p= 0.02). The modified EUSTAR index score decreased from 2.54 ± 1.8 at baseline to 0.49 ± 0.5 at 2 years (p= 0.02). Survival rate was 92.3% at 2 years, and 76.9% at 5 years. No unexpected adverse events were observed. Conclusions: In SSc patients undergoing LuTx, an excellent 2-year survival was observed, without any disease-related adverse events. Our study supports LuTx as a viable option in SSc patients with end-stage lung disease. Apart from expected recovery of lung function, LuTx was associated with improvement of mRSS and global systemic disease activity. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| Relation: | info:eu-repo/semantics/altIdentifier/pmid/39298492; info:eu-repo/semantics/altIdentifier/wos/WOS:001317896200001; journal:RHEUMATOLOGY; https://hdl.handle.net/2434/1100088 |
| DOI: | 10.1093/rheumatology/keae479 |
| الاتاحة: | https://hdl.handle.net/2434/1100088 https://doi.org/10.1093/rheumatology/keae479 |
| Rights: | info:eu-repo/semantics/restrictedAccess |
| رقم الانضمام: | edsbas.6548AB8 |
| قاعدة البيانات: | BASE |
| DOI: | 10.1093/rheumatology/keae479 |
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