title: Lung Transplant Outcomes in Systemic Sclerosis with Significant Esophageal Dysfunction. A Comprehensive Single-Center Experience.
abstract: Consideration of lung transplantation in patients with systemic sclerosis (SSc) remains guarded, often due to the concern for esophageal dysfunction and the associated potential for allograft injury and suboptimal post-lung transplantation outcomes.
  
  
  
  The purpose of this study was to systematically report our single-center experience regarding lung transplantation in the setting of SSc, with a particular focus on esophageal dysfunction.
  
  
  
  We retrospectively reviewed all lung transplants at our center from January 1, 2000 through August 31, 2012 (n = 562), comparing the SSc group (n = 35) to the following lung transplant diagnostic subsets: all non-SSc (n = 527), non-SSc diffuse fibrotic lung disease (n = 264), and a non-SSc matched group (n = 109). We evaluated post-lung transplant outcomes, including survival, primary graft dysfunction, acute rejection, bronchiolitis obliterans syndrome, and microbiology of respiratory isolates. In addition, we defined severe esophageal dysfunction using esophageal manometry and esophageal morphometry criteria on the basis of chest computed tomography images. For patients with SSc referred for lung transplant but subsequently denied (n = 36), we queried the reason(s) for denial with respect to the concern for esophageal dysfunction.
  
  
  
  The 1-, 3-, and 5-year post-lung transplant survival for SSc was 94, 77, and 70%, respectively, and similar to the other groups. The remaining post-lung transplant outcomes evaluated were also similar between SSc and the other groups. Approximately 60% of the SSc group had severe esophageal dysfunction. Pre-lung transplant chest computed tomography imaging demonstrated significantly abnormal esophageal morphometry for SSc when compared with the matched group. Importantly, esophageal dysfunction was the sole reason for lung transplant denial in a single case.
  
  
  
  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.
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: Catherine H
authors.identifier: 0000-0003-1307-2364
authors.initials: CH
authors.lastname: Miele
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: Kristin
authors.initials: K
authors.lastname: Schwab
authors.affiliation: 2 Department of Pulmonary and Critical Care Medicine, Banner University Medical Center, Phoenix, Arizona.
authors.forename: Rajeev
authors.initials: R
authors.lastname: Saggar
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|3 Department of Medicine Statistics Core.
authors.forename: Erin
authors.initials: E
authors.lastname: Duffy
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|3 Department of Medicine Statistics Core.
authors.forename: David
authors.initials: D
authors.lastname: Elashoff
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|3 Department of Medicine Statistics Core.
authors.forename: Chi-Hong
authors.initials: CH
authors.lastname: Tseng
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Sam
authors.initials: S
authors.lastname: Weigt
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: Deepshikha
authors.initials: D
authors.lastname: Charan
authors.affiliation: 6 Department of Radiology.
authors.forename: Fereidoun
authors.initials: F
authors.lastname: Abtin
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Jimmy
authors.initials: J
authors.lastname: Johannes
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Ariss
authors.initials: A
authors.lastname: Derhovanessian
authors.affiliation: 7 Department of Gastroenterology, and.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Conklin
authors.affiliation: 7 Department of Gastroenterology, and.
authors.forename: Kevin
authors.initials: K
authors.lastname: Ghassemi
authors.affiliation: 8 Department of Medicine, Scleroderma Program in the Division of Rheumatology, University of Michigan Health System, Ann Arbor, Michigan.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.affiliation: 9 Department of Surgery, University of Maryland School of Medicine, Baltimore Maryland; and.
authors.forename: Osama
authors.initials: O
authors.lastname: Siddiqui
authors.affiliation: 5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Abbas
authors.initials: A
authors.lastname: Ardehali
authors.affiliation: 5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Curtis
authors.initials: C
authors.lastname: Hunter
authors.affiliation: 5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Murray
authors.initials: M
authors.lastname: Kwon
authors.affiliation: 5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Reshma
authors.initials: R
authors.lastname: Biniwale
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: Michelle
authors.initials: M
authors.lastname: Lo
authors.affiliation: 10 Department of Rheumatology, University of California Los Angeles Medical Center, Los Angeles, California.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Volkmann
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: David
authors.initials: D
authors.lastname: Torres Barba
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: John A
authors.initials: JA
authors.lastname: Belperio
authors.affiliation: 4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: David
authors.identifier: 0000-0001-7021-8273
authors.initials: D
authors.lastname: Sayah
authors.affiliation: 11 Department of Medicine, Kaiser Permanente, Los Angeles, California.
authors.forename: Thomas
authors.initials: T
authors.lastname: Mahrer
authors.affiliation: 10 Department of Rheumatology, University of California Los Angeles Medical Center, Los Angeles, California.
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.affiliation: 10 Department of Rheumatology, University of California Los Angeles Medical Center, Los Angeles, California.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Kafaja
authors.affiliation: 8 Department of Medicine, Scleroderma Program in the Division of Rheumatology, University of Michigan Health System, Ann Arbor, Michigan.
authors.forename: Philip
authors.initials: P
authors.lastname: Clements
authors.affiliation: 4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Michael
authors.initials: M
authors.lastname: Shino
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: Aric
authors.initials: A
authors.lastname: Gregson
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.
authors.forename: Bernard
authors.initials: B
authors.lastname: Kubak
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.
authors.forename: Joseph P
authors.initials: JP
authors.lastname: Lynch
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: David
authors.initials: D
authors.lastname: Ross
authors.affiliation: 1 Department of Internal Medicine, David Geffen School of Medicine at the University of California Los Angeles.|4 Department of Pulmonary and Critical Care Medicine.|5 Lung and Heart-Lung Transplant and Pulmonary Hypertension Programs.
authors.forename: Rajan
authors.initials: R
authors.lastname: Saggar
mesh_terms: D000368:Aged; D001989:Bronchiolitis Obliterans / Q000209:etiology; D004935:Esophageal Diseases / Q000453:epidemiology* / Q000382:microbiology; D004947:Esophagus / Q000503:physiopathology; D005260:Female; D006085:Graft Survival; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011183:Postoperative Complications / Q000453:epidemiology*; D055031:Primary Graft Dysfunction / Q000209:etiology; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality* / Q000601:surgery*; D016019:Survival Analysis; D013997:Time Factors; D014481:United States
keywords: acute rejection; esophageal dysfunction; gastroesophageal reflux; lung transplantation; scleroderma