title: Survival and outcomes after lung transplantation for non-scleroderma connective tissue-related interstitial lung disease.
abstract: BACKGROUND
  Patients with non-scleroderma connective tissue-related lung disease (NS-CTLD), including rheumatoid arthritis, idiopathic inflammatory myopathies, Sjögren syndrome, mixed connective tissue disease, and systemic lupus erythematosus, may be at risk for worse outcomes after lung transplantation because of immune dysregulation or extrapulmonary manifestations of their underlying disease. We compared survival, acute and chronic rejection, and extrapulmonary organ dysfunction after transplantation in patients with NS-CTLD and idiopathic pulmonary fibrosis (IPF).
  
  
  METHODS
  This was a retrospective cohort study of patients with NS-CTLD and IPF who were listed in the Scientific Registry of Transplant Recipients and underwent lung transplantation from May 5, 2005, to March 1, 2016.
  
  
  RESULTS
  Patients with NS-CTLD (n = 275) were younger, a higher percentage female and non-white than patients with IPF (n = 6,346). NS-CTLD patients did not have worse adjusted survival (hazard ratio, 1.14, 95% confidence interval [CI], 0.92-1.42; p = 0.24). They were not more likely to have an episode of acute cellular rejection (odds ratio, 0.96; 95% CI, 0.72-1.28; p = 0.77) or to develop bronchiolitis obliterans syndrome (odds ratio, 0.82; 95% CI, 0.60-1.12; p = 0.21). Patients with NS-CTLD were not more likely to require plasmapheresis or dialysis or to develop a lymphoproliferative malignancy or liver disease after transplantation.
  
  
  CONCLUSIONS
  We found no significant differences in survival, acute or chronic rejection, or extrapulmonary organ dysfunction in patients who underwent lung transplantation for NS-CTLD compared with IPF. In appropriately selected candidates, NS-CTLD should not be considered a contraindication to lung transplantation.
authors.affiliation: Division of Pulmonary and Critical Care, Brigham and Women's Hospital, Boston, Massachusetts; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Courtwright
authors.affiliation: Division of Pulmonary and Critical Care, Brigham and Women's Hospital, Boston, Massachusetts; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts.
authors.forename: Souheil
authors.initials: S
authors.lastname: El-Chemaly
authors.affiliation: Division of Pulmonary and Critical Care, Brigham and Women's Hospital, Boston, Massachusetts; Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts.
authors.forename: Paul F
authors.initials: PF
authors.lastname: Dellaripa
authors.affiliation: Division of Pulmonary and Critical Care, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania; Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address: hjgoldberg@partners.org.
authors.forename: Hilary J
authors.initials: HJ
authors.lastname: Goldberg
mesh_terms: D000328:Adult; D000368:Aged; D003240:Connective Tissue Diseases / Q000150:complications* / Q000401:mortality / Q000601:surgery; D005260:Female; D006084:Graft Rejection; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000401:mortality* / Q000601:surgery*; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
keywords: extrapulmonary organ dysfunction; idiopathic pulmonary fibrosis; immune dysregulation; lung transplant; non-scleroderma connective tissue disease; outcomes