title: Survival of adults with systemic sclerosis following lung transplantation: a nationwide cohort study.
abstract: OBJECTIVE
  Many transplant programs are hesitant to offer lung transplantation to patients with systemic sclerosis (SSc) due to concerns about extrapulmonary involvement that might affect survival. The aim of this study was to determine whether adults with SSc have higher 1-year mortality rates after lung transplantation compared to those with interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) not due to SSc.
  
  
  METHODS
  Using data provided by the United Network for Organ Sharing, we performed a retrospective cohort study of 229 adults with SSc, 201 with PAH, and 3,333 with ILD who underwent lung transplantation in the US between May 4, 2005 and September 14, 2012. We examined associations between diagnosis and 1-year survival after lung transplantation using stratified Cox models adjusted for recipient, donor, and procedure factors.
  
  
  RESULTS
  Adults with SSc undergoing lung transplantation in the US had a multivariable-adjusted 48% relative increase in the 1-year mortality rate compared to those with non-SSc-related ILD (hazard ratio 1.48 [95% confidence interval 1.01-2.17]). However, we did not detect a difference in the risk of death at 1 year between those with SSc and those with non-SSc-related PAH (hazard ratio 0.85 [95% confidence interval 0.50-1.44]).
  
  
  CONCLUSIONS
  A diagnosis of SSc may confer an increased risk of death 1 year following lung transplantation compared to a diagnosis of ILD, but this risk is similar to that of PAH, a widely accepted indication for lung transplantation. Future work should identify modifiable risk factors that can improve transplant outcomes in this population.
authors.affiliation: Columbia University College of Physicians and Surgeons, New York, New York.
authors.forename: Elana J
authors.initials: EJ
authors.lastname: Bernstein
authors.forename: Eric R
authors.initials: ER
authors.lastname: Peterson
authors.forename: Jessica L
authors.initials: JL
authors.lastname: Sell
authors.forename: Frank
authors.initials: F
authors.lastname: D'Ovidio
authors.forename: Selim M
authors.initials: SM
authors.lastname: Arcasoy
authors.forename: Joan M
authors.initials: JM
authors.lastname: Bathon
authors.forename: David J
authors.initials: DJ
authors.lastname: Lederer
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000601:surgery*; D017563:Lung Diseases, Interstitial / Q000401:mortality / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate