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Lung Transplant Outcomes in Patients With Pulmonary Fibrosis With Telomere-Related Gene Variants.

Publication ,  Journal Article
Swaminathan, AC; Neely, ML; Frankel, CW; Kelly, FL; Petrovski, S; Durheim, MT; Bush, E; Snyder, L; Goldstein, DB; Todd, JL; Palmer, SM
Published in: Chest
September 2019

BACKGROUND: Pulmonary fibrosis (PF) is the most common disease indication for lung transplantation. Our recent work implicated an excess of rare genetic variants in the telomere-related genes TERT, RTEL1, and PARN in PF disease risk. The impact of such variants on posttransplant outcomes is uncertain. The objective of this study was to determine if patients with these PF-associated variants have altered rates of posttransplant acute rejection (AR), chronic lung allograft dysfunction (CLAD), and survival. METHODS: The study cohort consisted of 262 PF lung transplant recipients previously genetically characterized by whole exome sequencing. Thirty-one patients (11.8%) had variants in TERT, RTEL1, or PARN, whereas 231 (88.2%) did not. Multivariate Cox proportional hazards models adjusted for relevant clinical variables were used to assess the outcomes of death and CLAD. The AR burden was quantified and compared over the first posttransplant year. RESULTS: Patients with PF with disease-associated variants in TERT, RTEL1, or PARN had a significantly higher risk of death (adjusted hazard ratio [HR], 1.82; 95% CI, 1.07-3.08; P = .03) and CLAD (adjusted HR, 2.88; 95% CI, 1.42-5.87; P = .004) than patients without these variants. There was no difference in AR burden or rates of grade 3 primary graft dysfunction between the two groups. CONCLUSIONS: Rare variants in the telomere-related genes TERT, RTEL1, or PARN are associated with poor posttransplant outcomes among PF lung transplant recipients. Further research is needed to understand the biological mechanisms by which telomere-related variants increase the risk for death and CLAD.

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Published In

Chest

DOI

EISSN

1931-3543

Publication Date

September 2019

Volume

156

Issue

3

Start / End Page

477 / 485

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Telomere
  • Telomerase
  • Survival Rate
  • Respiratory System
  • Pulmonary Fibrosis
  • Primary Graft Dysfunction
  • Middle Aged
  • Male
  • Lung Transplantation
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Swaminathan, A. C., Neely, M. L., Frankel, C. W., Kelly, F. L., Petrovski, S., Durheim, M. T., … Palmer, S. M. (2019). Lung Transplant Outcomes in Patients With Pulmonary Fibrosis With Telomere-Related Gene Variants. Chest, 156(3), 477–485. https://doi.org/10.1016/j.chest.2019.03.030
Swaminathan, Aparna C., Megan L. Neely, Courtney W. Frankel, Fran L. Kelly, Slavé Petrovski, Michael T. Durheim, Erika Bush, et al. “Lung Transplant Outcomes in Patients With Pulmonary Fibrosis With Telomere-Related Gene Variants.Chest 156, no. 3 (September 2019): 477–85. https://doi.org/10.1016/j.chest.2019.03.030.
Swaminathan AC, Neely ML, Frankel CW, Kelly FL, Petrovski S, Durheim MT, et al. Lung Transplant Outcomes in Patients With Pulmonary Fibrosis With Telomere-Related Gene Variants. Chest. 2019 Sep;156(3):477–85.
Swaminathan, Aparna C., et al. “Lung Transplant Outcomes in Patients With Pulmonary Fibrosis With Telomere-Related Gene Variants.Chest, vol. 156, no. 3, Sept. 2019, pp. 477–85. Pubmed, doi:10.1016/j.chest.2019.03.030.
Swaminathan AC, Neely ML, Frankel CW, Kelly FL, Petrovski S, Durheim MT, Bush E, Snyder L, Goldstein DB, Todd JL, Palmer SM. Lung Transplant Outcomes in Patients With Pulmonary Fibrosis With Telomere-Related Gene Variants. Chest. 2019 Sep;156(3):477–485.

Published In

Chest

DOI

EISSN

1931-3543

Publication Date

September 2019

Volume

156

Issue

3

Start / End Page

477 / 485

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Telomere
  • Telomerase
  • Survival Rate
  • Respiratory System
  • Pulmonary Fibrosis
  • Primary Graft Dysfunction
  • Middle Aged
  • Male
  • Lung Transplantation