title: Outcomes in systemic sclerosis-related lung disease after lung transplantation.
abstract: BACKGROUND
  Lung disease is the leading cause of death in systemic sclerosis (SSc). The diagnosis of SSc-related lung disease (SSc-LD) is often a contraindication to lung transplantation (LT) due to concerns that extrapulmonary involvement will yield worse outcomes. We sought to evaluate posttransplantation outcomes in persons with SSc-LD with esophageal involvement compared with persons with nonconnective tissue disease-related interstitial lung disease (nCTD-ILD).
  
  
  METHODS
  From 1998 to 2012, persons undergoing LT for SSc-LD were age and gender matched in a 2:1 fashion to controls undergoing LT for nCTD-ILD. Esophageal function was assessed by pH testing and manometry. We defined esophageal dysfunction as the presence of a DeMeester score >14 or dysmotility more severe than "mild nonspecific disorder". The primary outcome was posttransplantation survival. Secondary outcomes included freedom from bronchiolitis obliterans syndrome (fBOS) and rates of acute rejection. Survival and fBOS were estimated with Kaplan-Meier methods. Acute rejection was compared with Student's t test.
  
  
  RESULTS
  Survival was similar in 23 persons with SSc-LD and 46 controls who underwent LT (P = 0.47). For the SSc-LD group, 1- and 5-year survival was 83% and 76% compared with 91% and 64% in the nCTD-ILD group, respectively. There were no differences in fBOS (P = 0.83). Rates of acute rejection were less in SSc-ILD (P = 0.05). Esophageal dysfunction was not associated with worse outcomes (P>0.55).
  
  
  CONCLUSIONS
  Persons with SSc-LD appear to have similar survival and fBOS as persons transplanted for nCTD-ILD. The risk of acute rejection after transplantation may be reduced in persons with SSc-LD. Esophageal involvement does not appear to impact outcomes.
authors.affiliation: Department of Internal Medicine, University of California-San Francisco, San Francisco, CA, USA.
authors.forename: Peter D
authors.initials: PD
authors.lastname: Sottile
authors.forename: David
authors.initials: D
authors.lastname: Iturbe
authors.forename: Tamiko R
authors.initials: TR
authors.lastname: Katsumoto
authors.forename: M Kari
authors.initials: MK
authors.lastname: Connolly
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
authors.forename: Lorriana A
authors.initials: LA
authors.lastname: Leard
authors.forename: Steven
authors.initials: S
authors.lastname: Hays
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Golden
authors.forename: Charles
authors.initials: C
authors.lastname: Hoopes
authors.forename: Jasleen
authors.initials: J
authors.lastname: Kukreja
authors.forename: Jonathan P
authors.initials: JP
authors.lastname: Singer
mesh_terms: D000208:Acute Disease; D000328:Adult; D000368:Aged; D001989:Bronchiolitis Obliterans / Q000209:etiology; D016009:Chi-Square Distribution; D015154:Esophageal Motility Disorders / Q000209:etiology; D005260:Female; D006084:Graft Rejection / Q000209:etiology; D006801:Humans; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000601:surgery*; D016040:Lung Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012495:San Francisco; D012595:Scleroderma, Systemic / Q000150:complications* / Q000401:mortality; D013997:Time Factors; D016896:Treatment Outcome
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