title: Survival and extrapulmonary course of connective tissue disease after lung transplantation.
abstract: BACKGROUND
  Connective tissue disease (CTD)-related lung dysfunction is a common cause of morbidity and mortality; however, few lung transplantations (LTs) are performed in this population secondary to uncertainty regarding the posttransplant survival, outcome, and management.
  
  
  OBJECTIVE
  The objectives were to evaluate the survival and the pulmonary and extrapulmonary courses of CTD after LT.
  
  
  METHODS
  Survival outcomes of patients documented within the Organ Procurement and Transplantation Network who had undergone a LT for CTD were compared with those who underwent LT for chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). In addition, the pulmonary and extrapulmonary courses of the CTD were evaluated after LT.
  
  
  RESULTS
  From 1991 to 2009, there were 284 documented LT in patients with CTD. Post-LT cumulative survival of patients with CTD was less than that for COPD through 5 years, with a difference that peaked at 1 year (72.7% vs. 83.1%, P < 0.001). When patients with CTD were compared with those with IPF, a difference was only noted at 1 year (72.7% vs. 77.7%, P = 0.049). There were no documented post-LT pulmonary recurrences of the CTD, and extrapulmonary flares of the CTD were rare (1 possible flare per 20.3 patient-years and 1 probable flare per 81.0 patient-years).
  
  
  CONCLUSIONS
  Cumulative survival of patients with CTD who underwent LT is similar to those with IPF and slightly less than those with COPD, with an increased risk of mortality that was most prominent at 6 months after transplant followed by subsequent narrowing of the survival differences over time. Lung transplantation may be a viable therapeutic option for patients with end-stage lung dysfunction resulting from a CTD.
authors.affiliation: Department of Rheumatology, Loyola University Medical Center, Louisville, KY 40207, USA. ttakagish@gmail.com
authors.forename: Troy
authors.initials: T
authors.lastname: Takagishi
authors.forename: Rochella
authors.initials: R
authors.lastname: Ostrowski
authors.forename: Charles
authors.initials: C
authors.lastname: Alex
authors.forename: Karen
authors.initials: K
authors.lastname: Rychlik
authors.forename: Karen
authors.initials: K
authors.lastname: Pelletiere
authors.forename: Rodney
authors.initials: R
authors.lastname: Tehrani
mesh_terms: D000328:Adult; D000368:Aged; D003240:Connective Tissue Diseases / Q000401:mortality* / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006084:Graft Rejection / Q000453:epidemiology; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000401:mortality / Q000601:surgery*; D015994:Incidence; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D029424:Pulmonary Disease, Chronic Obstructive / Q000401:mortality / Q000601:surgery*; D012008:Recurrence; D012042:Registries; D015996:Survival Rate; D016896:Treatment Outcome