Abstract
BACKGROUND: Scleroderma is an autoimmune disease affecting the skin and internal organs, with pulmonary disease being the leading cause of mortality. Lung transplantation is a potential therapy, but its indication has been limited by concerns about complications, such as esophageal dysmotility. METHODS: A retrospective analysis was performed on 959 lung transplant patients from 2011 to 2023, including 77 with scleroderma-related lung disease. Survival rates, post-transplant complications, such as chronic lung allograft dysfunction, and acute cellular rejection rates. RESULTS: Scleroderma patients had higher mean pulmonary arterial pressure (32 vs. 24 mmHg, p < 0.001) and increased esophageal dysmotility (85% vs. 27%, p < 0.001). Double lung transplantation was more common (99% vs. 87%, p = 0.003). Scleroderma patients experienced higher rates of delayed chest closure (44% vs. 25%, p < 0.001), severe primary graft dysfunction at 72 hours (30% vs. 17%, p = 0.006), and longer mechanical ventilation (median 7 vs. 4 days, p = 0.002). They also required more gastrojejunostomy tubes (79% vs. 20%, p < 0.001) and had longer ICU stays (median 12 vs. 8 days, p = 0.007). However, adjusted competing risks regression showed no significant association between scleroderma and chronic lung allograft dysfunction (HR 0.69 [0.33 - 1.46], p = 0.31) or survival (HR 0.90 [0.56 - 1.45], p = 0.68). CONCLUSIONS: Our findings suggest that lung transplantation might be an important therapeutic option for patients with scleroderma, showing outcomes similar to those of patients with different underlying conditions.