title: Lung Transplant in Patients with Scleroderma Compared with Pulmonary Fibrosis. Short- and Long-Term Outcomes.
abstract: Patients with advanced lung disease due to systemic sclerosis have long been considered suboptimal and often unacceptable candidates for lung transplant.
  
  
  
  To examine post-lung transplant survival of patients with systemic sclerosis compared with patients with pulmonary fibrosis and to identify risk factors for 1-year mortality.
  
  
  
  In a retrospective cohort study, we compared post-lung transplant outcomes of 72 patients with scleroderma with those of 311 patients with pulmonary fibrosis between June 2005 and September 2013 at our institution. Actuarial survival estimates were calculated using Kaplan-Meier curves. In Cox regression models, we determined risk factors for post-transplant mortality, controlling for whether patients had scleroderma or pulmonary fibrosis.
  
  
  
  Post-transplant survival did not differ significantly between scleroderma and pulmonary fibrosis at year 1 (81% scleroderma vs. 79% pulmonary fibrosis; P = 0.743), at year 5 conditional on 1-year survival (66% vs. 58%; P = 0.249), or overall (P = 0.385). In multivariate analysis, body mass index greater than or equal to 35 kg/m(2) predicted poor 1-year survival in pulmonary fibrosis (hazard ratio, 2.76; P = 0.003). Acute cellular rejection-free survival did not differ significantly between the scleroderma and pulmonary fibrosis cohorts. Patients with scleroderma had significantly better bronchiolitis obliterans syndrome stage 1 or higher-free survival than did patients with pulmonary fibrosis.
  
  
  
  Our findings that 1- and 5-year survival rates of patients with scleroderma were similar to those of patients with pulmonary fibrosis indicate that lung transplant is a reasonable treatment option in selected patients with scleroderma.
authors.affiliation: 1 Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine.
authors.forename: Maria M
authors.initials: MM
authors.lastname: Crespo
authors.affiliation: 2 Department of Cardiothoracic Surgery, University of Pennsylvania, Philadelphia, Pennsylvania; and.
authors.forename: Christian A
authors.initials: CA
authors.lastname: Bermudez
authors.affiliation: 3 Department of Psychiatry, University of Pittsburgh Medical Center and University of Pittsburgh, Pittsburgh, Pennsylvania.
authors.forename: Mary Amanda
authors.initials: MA
authors.lastname: Dew
authors.affiliation: 1 Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine.
authors.forename: Bruce A
authors.initials: BA
authors.lastname: Johnson
authors.affiliation: 1 Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine.
authors.forename: M Patricia
authors.initials: MP
authors.lastname: George
authors.affiliation: 4 Department of Cardiothoracic Surgery, University of Iowa, Iowa City, Iowa.
authors.forename: Jay
authors.initials: J
authors.lastname: Bhama
authors.affiliation: 1 Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine.
authors.forename: Matthew
authors.initials: M
authors.lastname: Morrell
authors.affiliation: 5 Division of Cardiothoracic Transplantation, Department of Cardiothoracic Surgery, and.
authors.forename: Jonathan
authors.initials: J
authors.lastname: D'Cunha
authors.affiliation: 5 Division of Cardiothoracic Transplantation, Department of Cardiothoracic Surgery, and.
authors.forename: Norihisa
authors.initials: N
authors.lastname: Shigemura
authors.affiliation: 5 Division of Cardiothoracic Transplantation, Department of Cardiothoracic Surgery, and.
authors.forename: Thomas J
authors.initials: TJ
authors.lastname: Richards
authors.affiliation: 1 Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Pilewski
mesh_terms: D000328:Adult; D015992:Body Mass Index; D001989:Bronchiolitis Obliterans / Q000209:etiology; D002423:Cause of Death; D005260:Female; D006084:Graft Rejection / Q000453:epidemiology*; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010414:Pennsylvania; D011183:Postoperative Complications / Q000453:epidemiology*; D011658:Pulmonary Fibrosis / Q000401:mortality* / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000401:mortality* / Q000601:surgery; D016019:Survival Analysis; D016896:Treatment Outcome
keywords: bronchiolitis obliterans syndrome; lung transplantation; outcomes; scleroderma; transplant rejection