Long-Term Survival in Bilateral Lung Transplantation for Scleroderma-Related Lung Disease

Background Lung disease is the leading cause of morbidity and death in scleroderma patients, but scleroderma is often considered a contraindication to lung transplantation because of concerns for worse outcomes. We evaluated whether 5-year survival in scleroderma patients after lung transplantation differed from other patients with restrictive lung disease. Methods This was a single-center, retrospective cohort study of all patients undergoing bilateral lung transplantation for scleroderma-related pulmonary disease between January 2006 and December 2014. This cohort was compared with patients undergoing bilateral lung transplantation for nonscleroderma group D restrictive disease. Primary outcomes reported were 1-year and 5-year survival. Diagnoses were identified by United Network of Organ Sharing listing and were confirmed by clinical examination and prelisting workup. Results We compared 26 patients who underwent BLT for scleroderma and 155 patients who underwent BLT for group D restrictive disease. Overall, the nonscleroderma cohort was younger, with lower lung allocation score but no difference in functional status. Donor characteristics were not different between the cohorts. Survival at 1 year was not different (73.1% vs 80.0%, p  = 0.323). Long-term survival at 5 years was also not significantly different (65.4% vs 66.5%, p  = 0.608). Multivariate Cox proportional hazards analysis found no differences in survival between scleroderma and nonscleroderma group D restrictive disease (hazard ratio, 2.19; p  = 0.122). Conclusions Despite being at high risk for extrapulmonary complications, patients undergoing bilateral lung transplantation for scleroderma have similar 1-year and 5-year survival as those with restrictive lung disease. Transplantation is a reasonable treatment option for a carefully selected population of candidates.