Short and Long Term Outcomes for Scleroderma Related Lung Transplantation

Abstract

PurposeHistorically, lung transplant for scleroderma-related failure has resulted in poor outcomes. We sought to determine if lung transplant in patients with scleroderma affected peri-operative complications and survival in the modern era.MethodsThe United Network for Organ Sharing database was queried from 2005- 2019 to include adult patients who underwent lung transplantation. Patients were excluded if they underwent multi-organ or re-transplant. Patients were then identified for a diagnosis of autoimmune disease and divided into two groups: scleroderma (S, n=401) and non-scleroderma (NS, n=1105). Outcomes were analyzed using idiopathic fibrosis as a surrogate. T-test and chi-square analysis was used to compare peri-operative outcomes. Survival was assessed using Kaplan-Meier analysis.ResultsThere was no difference in survival between S, NS, and all other indications for lung transplant (p=0.356, Figure 1). Short term outcomes demonstrated that a significantly higher number of patients with S were intubated at 72 hours post-transplantation (p=0.005). However, there was no difference in the use of inhaled nitric oxide, extracorporeal membrane oxygenation, PaO2, or FiO2 at 72 hours after transplantation between the two groups. The effect of pulmonary hypertension (PH) at the time of transplant was analyzed in patients with S (n=273) and pulmonary fibrosis not due to S (n=10,400). There was no significant difference in survival between these two groups (p=0.109). Finally, there was no difference in survival between S patients with (n=286) and without PH (n=90, p=0.527).ConclusionThe results of this analysis suggest that selected patients with scleroderma have equivalent survival outcomes when compared to patients with non-scleroderma autoimmune disease and other thoracic diagnoses. Despite a selection bias, the data support favorable outcomes after lung transplant in patients with scleroderma. Therefore, a diagnosis of scleroderma should not be a deterrent to lung transplantation. Historically, lung transplant for scleroderma-related failure has resulted in poor outcomes. We sought to determine if lung transplant in patients with scleroderma affected peri-operative complications and survival in the modern era. The United Network for Organ Sharing database was queried from 2005- 2019 to include adult patients who underwent lung transplantation. Patients were excluded if they underwent multi-organ or re-transplant. Patients were then identified for a diagnosis of autoimmune disease and divided into two groups: scleroderma (S, n=401) and non-scleroderma (NS, n=1105). Outcomes were analyzed using idiopathic fibrosis as a surrogate. T-test and chi-square analysis was used to compare peri-operative outcomes. Survival was assessed using Kaplan-Meier analysis. There was no difference in survival between S, NS, and all other indications for lung transplant (p=0.356, Figure 1). Short term outcomes demonstrated that a significantly higher number of patients with S were intubated at 72 hours post-transplantation (p=0.005). However, there was no difference in the use of inhaled nitric oxide, extracorporeal membrane oxygenation, PaO2, or FiO2 at 72 hours after transplantation between the two groups. The effect of pulmonary hypertension (PH) at the time of transplant was analyzed in patients with S (n=273) and pulmonary fibrosis not due to S (n=10,400). There was no significant difference in survival between these two groups (p=0.109). Finally, there was no difference in survival between S patients with (n=286) and without PH (n=90, p=0.527). The results of this analysis suggest that selected patients with scleroderma have equivalent survival outcomes when compared to patients with non-scleroderma autoimmune disease and other thoracic diagnoses. Despite a selection bias, the data support favorable outcomes after lung transplant in patients with scleroderma. Therefore, a diagnosis of scleroderma should not be a deterrent to lung transplantation.