Lung Transplant Outcomes in Systemic Sclerosis with Significant Esophageal Dysfunction. A Comprehensive Single-Center Experience

Abstract

Relative to other lung transplant indications, our SSc group experienced comparable survival, primary graft dysfunction, acute rejection, bronchiolitis obliterans syndrome, and microbiology of respiratory isolates, despite the high prevalence of severe esophageal dysfunction. Esophageal dysfunction rarely precluded active listing for lung transplantation.

Treatment of Systemic Sclerosis–related Interstitial Lung Disease: A Review of Existing and Emerging Therapies

“…Deemed a last-resort intervention for patients with end-stage lung disease, lung transplantation is presently performed at select transplantation centers for patients with SSc. The literature in this area is evolving, and although a retrospective cohort study found a 48% increase in the 1-year mortality rate among patients with SSc undergoing lung transplantation in the United States compared with those with non-SSc-ILD (100), other studies have not found any difference in survival after lung transplantation between patients with SSc and non-SSc patients with fibrotic lung diseases (101)(102)(103)(104). Factors associated with higher mortality in patients with SSc-ILD undergoing lung transplantation include older age (104) and elevated body mass index (103).…”

“…The literature in this area is evolving, and although a retrospective cohort study found a 48% increase in the 1-year mortality rate among patients with SSc undergoing lung transplantation in the United States compared with those with non-SSc-ILD (100), other studies have not found any difference in survival after lung transplantation between patients with SSc and non-SSc patients with fibrotic lung diseases (101)(102)(103)(104). Factors associated with higher mortality in patients with SSc-ILD undergoing lung transplantation include older age (104) and elevated body mass index (103). However, contrary to previous thought, severity of esophageal dysfunction by either morphometry or manometry criteria was not associated with survival in patients with SSc-ILD (n = 35) who underwent lung transplantation at a single center (104).…”

“…Factors associated with higher mortality in patients with SSc-ILD undergoing lung transplantation include older age (104) and elevated body mass index (103). However, contrary to previous thought, severity of esophageal dysfunction by either morphometry or manometry criteria was not associated with survival in patients with SSc-ILD (n = 35) who underwent lung transplantation at a single center (104). These findings are consistent with a prior single-center study, which found no association with the presence of esophageal dysfunction and survival (102).…”

Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease

“…The use of lung transplantation in patients with SSc requires discussion due to concerns over whether esophageal dysfunction and gastro-esophageal reflux may cause damage to the transplanted lung, and whether the multimorbidity of patients with SSc will impact outcomes. However, recent data suggest that post-transplant outcomes, including graft dysfunction and survival, are similar in selected patients with SSc-ILD as in those with other types of ILD [47][48][49].…”

Lung Transplantation in Scleroderma. Time for the Pendulum to Swing?

“…Miele and colleagues from the University of California at Los Angeles (UCLA) Lung Transplantation Program report their experience in 35 patients with SSc-ILD who underwent lung transplant between 2000 and 2012 (14). In addition to comparing outcomes to patients with diffuse fibrotic lung disease, the authors compared outcomes to all other lung transplant indications combined and to a 4:1 matched cohort based on age, lung allocation score, transplant type, and presence of pulmonary hypertension.…”