Clinical Predictors of Lung Transplant Outcomes in Patients with Scleroderma Compared with Pulmonary Fibrosis

Purpose Primary Graft Disfunction (PGD) is the leading cause of early morbidity and mortality after lung transplant. We sought to identify risk factors for PGD and survival in patients with scleroderma undergoing lung transplant compared to patients with pulmonary fibrosis. Methods This was a single center retrospective cohort study, of patients that underwent lung transplantation for scleroderma or pulmonary fibrosis from 2007 to 2020. Propensity scores were used to match the scleroderma group with the pulmonary fibrosis group based on lung allocation score and age at lung transplant. Univariable analyses identified factors associated with the development of PGD 72 hours posttransplant, and six-month survival. Survival rates were compared using Kaplan-Meier analysis. Results We compared 104 patients with scleroderma to 389 patients with pulmonary fibrosis who underwent lung transplant. After propensity matching, compared with pulmonary fibrosis, scleroderma patients had reduced 90-day survival (99.0% vs 90.2%, p=0.007). However, there was no statistically significant difference in 1 year (84.4% vs 79.1%, p=0.23) or 5-year survival (64.9% vs 52.9%, p=0.98). Scleroderma patients were more likely to have PGD 72 hours posttransplant (29.4% vs 11.8%, p=0.01). Scleroderma patients who developed PGD had higher volumes of intraoperative transfusion of blood products (p=0.02), plasma (p=0.04), platelets (p=0.03), but not red blood cells (p=0.28). Preoperative history of chronic kidney disease (p<0.001) and postoperative Acute Kidney Injury [AKI] (p=0.028) were also associated with the development of PGD. Donor factors for developing PGD in scleroderma patients were traumatic brain injury as cause of donor death (p=0.015) and donor history of smoking (p=0.02). Risk factors for 6-month mortality were postoperative right ventricular dysfunction (p=0.02) or dilation (p=0.003), stroke (p=0.02), atrial fibrillation (p=0.01), PGD (p<0.001), AKI (p=0.005) and decreased glomerular filtration rate 3 months posttransplant (p=0.04). Conclusion Scleroderma had higher rates of post lung transplant PGD compared to pulmonary fibrosis. Kidney function plays a prominent role in the risk of developing PGD and mortality in this population. Despite reduced 90-day posttransplant survival in scleroderma patients, 1- and 5-year survival rates were similar. Primary Graft Disfunction (PGD) is the leading cause of early morbidity and mortality after lung transplant. We sought to identify risk factors for PGD and survival in patients with scleroderma undergoing lung transplant compared to patients with pulmonary fibrosis. This was a single center retrospective cohort study, of patients that underwent lung transplantation for scleroderma or pulmonary fibrosis from 2007 to 2020. Propensity scores were used to match the scleroderma group with the pulmonary fibrosis group based on lung allocation score and age at lung transplant. Univariable analyses identified factors associated with the development of PGD 72 hours posttransplant, and six-month survival. Survival rates were compared using Kaplan-Meier analysis. We compared 104 patients with scleroderma to 389 patients with pulmonary fibrosis who underwent lung transplant. After propensity matching, compared with pulmonary fibrosis, scleroderma patients had reduced 90-day survival (99.0% vs 90.2%, p=0.007). However, there was no statistically significant difference in 1 year (84.4% vs 79.1%, p=0.23) or 5-year survival (64.9% vs 52.9%, p=0.98). Scleroderma patients were more likely to have PGD 72 hours posttransplant (29.4% vs 11.8%, p=0.01). Scleroderma patients who developed PGD had higher volumes of intraoperative transfusion of blood products (p=0.02), plasma (p=0.04), platelets (p=0.03), but not red blood cells (p=0.28). Preoperative history of chronic kidney disease (p<0.001) and postoperative Acute Kidney Injury [AKI] (p=0.028) were also associated with the development of PGD. Donor factors for developing PGD in scleroderma patients were traumatic brain injury as cause of donor death (p=0.015) and donor history of smoking (p=0.02). Risk factors for 6-month mortality were postoperative right ventricular dysfunction (p=0.02) or dilation (p=0.003), stroke (p=0.02), atrial fibrillation (p=0.01), PGD (p<0.001), AKI (p=0.005) and decreased glomerular filtration rate 3 months posttransplant (p=0.04). Scleroderma had higher rates of post lung transplant PGD compared to pulmonary fibrosis. Kidney function plays a prominent role in the risk of developing PGD and mortality in this population. Despite reduced 90-day posttransplant survival in scleroderma patients, 1- and 5-year survival rates were similar.