The diagnosis of SSc was made by rheumatologists based on the American College of Rheumatology (ACR) criteria of 1980 or the ACR/European League Against Rheumatism criteria of 2013 14, 15. Using a lung transplant candidate database, patients with SSc-ILD who were registered for deceased-donor lung transplantation at the Kyoto University Hospital between April 2010 and April 2022 were identified. Therefore, this study aimed to assess the prognosis of patients with SSc-ILD on the lung transplant waiting list and evaluate post-transplant outcomes for patients attending a lung transplant center in Asia. However, there are no reports of lung transplantation in patients with SSc-ILD from non-Western countries. The 2021 International Society for Heart and Lung Transplantation (ISHLT) consensus document proposed that lung transplantation is an acceptable treatment for selected patients with CTD, including advanced SSc-ILD 13. Although recent studies have shown similar outcomes for lung transplantation in patients with IPF and SSc-ILD, more research is needed to determine the safety and efficacy of the transplant approach for SSc-ILD 9, 10, 11, 12. This is due to concern for worse post-transplant outcomes associated with extrapulmonary CTD manifestations 7, 8. However, several centers have been reluctant to offer lung transplants to patients with SSc-ILD and other connective tissue disease (CTD)-associated ILD (CTD-ILD). Idiopathic pulmonary fibrosis (IPF), a progressive type of ILD, is a widely accepted indication for lung transplantation 7. Lung transplantation is a potentially life-saving intervention for selected patients with severe ILD refractory to medical therapy 6, 7. Although recent studies have shown that several immunosuppressants, such as cyclophosphamide, mycophenolate mofetil, and nintedanib (an anti-fibrotic agent), can reduce the rate of decline in forced vital capacity (FVC) in progressive SSc-ILD, there is no evidence-based pharmacological therapy that improves survival and reverses extensive lung damage 3, 4, 5. SSc-associated ILD (SSc-ILD) is a mortality risk factor for patients with SSc 2. Interstitial lung disease (ILD) is a major complication and the leading cause of death in SSc, accounting for 35% of SSc-related deaths 1. Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by microangiopathy and fibrosis affecting the skin and visceral organs. The higher post-transplant survival rate at our institute than previously reported suggests that lung transplantation is acceptable in Asian patients with SSc-ILD. The 5-year survival rate for post-transplant patients with SSc-ILD was 86.2%. Analysis of 15 recipients showed improved forced vital capacity with a median of 55.1% at baseline, 65.8% at 6 months, and 80.3% at 12 months post-transplant. Median duration from registration to deceased-donor LT was 28.9 months and that from registration to living-donor LT or death was 6.5 months. Ten patients received deceased-donor LT (34%), two received living-donor LT (7%), seven died waiting for LT (24%), and ten survived on the waiting list (34%). We investigated post-transplant outcomes in recipients who underwent LT for SSc-ILD, between February 2002 and April 2022. In this single-center retrospective study, 29 patients with SSc-ILD, registered for deceased LT at Kyoto University Hospital, between 20, were identified. There is limited data on lung transplantation outcomes in patients with SSc-ILD, in non-Western populations.We assessed survival data of patients with SSc-ILD, on the lung transplant (LT) waiting list, and evaluated post-transplant outcomes in patients from an Asian LT center. Advanced systemic sclerosis-associated interstitial lung disease (SSc-ILD) can be treated with lung transplantation.
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