Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study

Elana J. Bernstein, Eric R. Peterson, Jessica L. Sell, Frank D'Ovidio, Selim M. Arcasoy, Joan M. Bathon, David J. Lederer

Research output: Contribution to journalArticle

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]). Conclusion 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.

Original languageEnglish (US)
Pages (from-to)1314-1322
Number of pages9
JournalArthritis and Rheumatology
Volume67
Issue number5
DOIs
StatePublished - May 1 2015
Externally publishedYes

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Lung Transplantation
Systemic Scleroderma
Cohort Studies
Interstitial Lung Diseases
Survival
Pulmonary Hypertension
Sclerosis
Confidence Intervals
Transplants
Mortality
Proportional Hazards Models
Retrospective Studies
Tissue Donors
Population

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Rheumatology
  • Medicine(all)

Cite this

Bernstein, E. J., Peterson, E. R., Sell, J. L., D'Ovidio, F., Arcasoy, S. M., Bathon, J. M., & Lederer, D. J. (2015). Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study. Arthritis and Rheumatology, 67(5), 1314-1322. https://doi.org/10.1002/art.39021

Survival of adults with systemic sclerosis following lung transplantation : A nationwide cohort study. / Bernstein, Elana J.; Peterson, Eric R.; Sell, Jessica L.; D'Ovidio, Frank; Arcasoy, Selim M.; Bathon, Joan M.; Lederer, David J.

In: Arthritis and Rheumatology, Vol. 67, No. 5, 01.05.2015, p. 1314-1322.

Research output: Contribution to journalArticle

Bernstein, EJ, Peterson, ER, Sell, JL, D'Ovidio, F, Arcasoy, SM, Bathon, JM & Lederer, DJ 2015, 'Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study', Arthritis and Rheumatology, vol. 67, no. 5, pp. 1314-1322. https://doi.org/10.1002/art.39021
Bernstein EJ, Peterson ER, Sell JL, D'Ovidio F, Arcasoy SM, Bathon JM et al. Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study. Arthritis and Rheumatology. 2015 May 1;67(5):1314-1322. https://doi.org/10.1002/art.39021
Bernstein, Elana J. ; Peterson, Eric R. ; Sell, Jessica L. ; D'Ovidio, Frank ; Arcasoy, Selim M. ; Bathon, Joan M. ; Lederer, David J. / Survival of adults with systemic sclerosis following lung transplantation : A nationwide cohort study. In: Arthritis and Rheumatology. 2015 ; Vol. 67, No. 5. pp. 1314-1322.
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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]). Conclusion 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.",
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N2 - 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]). Conclusion 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.

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