2047: Trends in Lung Transplant Listing Outcomes in Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) Across Two Decades

Background/Purpose: Lung transplantation represents a potential life-extending therapy for patients with advanced CTD-ILD. This study aims to characterize lung transplant listing outcomes among CTD-ILD patients over a 20-year period using the Organ Procurement and Transplantation Network (OPTN) national database.

Methods: Data analyzed from the OPTN between 2003-2023 included adults ≥18 years of age with CTD-ILD listed for lung transplantation. Patients were categorized into six subgroups based on diagnosis: scleroderma, lupus, rheumatoid arthritis (RA), myositis, Sjögren’s, and "Other" (including mixed connective tissue disease, CTD, etc.). Trends in listing and outcomes were analyzed in 5-year quartiles and across diagnoses. We analyzed the proportion of patients getting successfully transplanted, wait list times, and death on transplant list.

Results: A total of 1,977 individuals with CTD-ILD were listed for lung transplantation between 2003-2023.


Trends across time: In 5-year intervals numbers of patients listed for transplant increased from 185 in 2003-2007, 443 in 2008-2013, 605 in 2014-2018, and 744 in 2019-2023. The proportion of patients who underwent successful transplantation also steadily increased: from 62.7% in 2003-2008, 71.6% in 2009-2013, 75.9% in 2014-2018, and 81.6% in 2019-2023. The average time from listing to transplant in days decreased significantly, from 233.9 in 2003-2008 to 120.9 in 2019-2023. Death on the waitlist decreased over time: from 19.5% in 2003-2008, 12.2% in 2009-2013, 7.9% in 2014-2018, and 5% in 2019-2023.


Trends across diseases: Of the diagnosis groups listed for transplant, Scleroderma was the most common (47%, n=924) followed by “Other” (16%, n=323), RA (15%, n=293), myositis (11%, n=221), Sjögren’s (7%, n=137), and lupus (4%, n=79). Rates of proceeding to transplantation were similar: “Other” (78.32%, n=253), Sjögren’s (77.37%, n=106), RA (77.13%, n=226), myositis (76.47%, n=169), scleroderma (74.35%, n=687), and lupus (73.41%, n=58). Mean days from listing to transplant ranged from: lupus (205 days), Sjogren's (199 days), scleroderma (165 days), other (163 days), myositis (154 days), and RA (119 days). Death while awaiting transplant ranged from Scleroderma (11%, n=101), Sjogren’s (9.5%, n=13), RA (8.5%, n=25), myositis (6.8%, n=15), other (5.3%, n=17), and lupus (5%, n=4).

Conclusion: Our study suggests positive trends in lung transplant listing outcomes for CTD-ILD as reflected by an increase in the absolute number of listings and improved rates of transplantation. This is matched by shorter wait times and reduced wait list mortality. All CTD diagnoses seem equally likely to get a lung transplant. Some disease specific variability was noted in wait list times. Scleroderma patients exhibit slightly higher waitlist mortality. Limitations in OPTN registry data may impact our results.