Outcomes of Single Lung Transplantation for Connective Tissue Disease–Associated Interstitial Lung Disease: A Single-Center Retrospective Comparison with Idiopathic Pulmonary Fibrosis

Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a serious manifestation of systemic autoimmune disorders and may progress to end-stage respiratory failure.Although lung transplantation represents a potential life-prolonging treatment, outcome data for CTD-ILD remain limited, particularly in single lung transplantation recipients.We conducted a single-center retrospective cohort study to clarify post-transplant outcomes in adult patients who underwent single lung transplantation from brain-dead donors for CTD-ILD, using idiopathic pulmonary fibrosis (IPF) as a comparator.Between January 2001 and March 2024, a total of 29 single lung transplantations were performed for these patients (CTD-ILD, n = 13; IPF, n = 16).One-year overall survival was 92.3% in the CTD-ILD group and 87.5% in the IPF group, and five-year overall survival was 63.5% and 61.9%, respectively, with no significant difference observed throughout the observation period (log-rank test, p = 0.903).Freedom from chronic lung allograft dysfunction (CLAD) was significantly lower in the CTD-ILD group than in the IPF group (p = 0.048), with no CLAD cases found in the IPF group in this cohort.However, five-year CLAD-free survival did not differ significantly between groups (p = 0.634).All cases of CLAD in the CTD-ILD group were classified as restrictive allograft syndrome (RAS).In a real-world Japanese clinical setting, single lung transplantation for appropriately selected patients with CTD-ILD achieved overall survival comparable to that for IPF.The high incidence of RAS among CTD-ILD recipients observed in our cohort merits