title: Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension.
abstract: OBJECTIVE
  Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention.
  
  
  METHODS
  Survival following lung transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters.
  
  
  RESULTS
  During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52, 95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at approximately 64%, among all 3 groups.
  
  
  CONCLUSIONS
  Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.
authors.affiliation: Johns Hopkins University, Baltimore, Maryland 21224, USA.
authors.forename: Lionel
authors.initials: L
authors.lastname: Schachna
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Medsger
authors.forename: James H
authors.initials: JH
authors.lastname: Dauber
authors.forename: Fredrick M
authors.initials: FM
authors.lastname: Wigley
authors.forename: Neil A
authors.initials: NA
authors.lastname: Braunstein
authors.forename: Barbara
authors.initials: B
authors.lastname: White
authors.forename: Virginia D
authors.initials: VD
authors.lastname: Steen
authors.forename: John V
authors.initials: JV
authors.lastname: Conte
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Yang
authors.forename: Kenneth R
authors.initials: KR
authors.lastname: McCurry
authors.forename: Marvin C
authors.initials: MC
authors.lastname: Borja
authors.forename: David E
authors.initials: DE
authors.lastname: Plaskon
authors.forename: Jonathan B
authors.initials: JB
authors.lastname: Orens
authors.forename: Allan C
authors.initials: AC
authors.lastname: Gelber
mesh_terms: D000328:Adult; D005260:Female; D006785:Hospitals, University; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008396:Maryland / Q000453:epidemiology; D008875:Middle Aged; D010414:Pennsylvania / Q000453:epidemiology; D011651:Pulmonary Artery / Q000473:pathology*; D011658:Pulmonary Fibrosis / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate