title: Lung transplantation for respiratory failure resulting from systemic disease.
abstract: BACKGROUND
  Lung transplantation for pulmonary failure resulting from systemic disease is controversial. We reviewed our transplant experience in patients with sarcoidosis, scleroderma, lymphangioleiomyomatosis, and graft-versus-host disease.
  
  
  METHODS
  This retrospective review examined the outcome of 23 patients who underwent pulmonary transplantation for these systemic diseases. Group 1 included 15 patients with pulmonary hypertension who underwent transplantation (9 for sarcoidosis, 6 for scleroderma), and group 2 included 8 patients with normal pulmonary artery pressures who underwent transplantation (5 for lymphangioleiomyomatosis, 3 for graft-versus-host disease). The incidences of infection and rejection, pulmonary function, and survival were measured and compared with those of patients who underwent transplantation for isolated pulmonary disease.
  
  
  RESULTS
  Although there were no differences in the rate of infection between patients who underwent transplantation for systemic versus isolated disease, patients with pulmonary hypertension who underwent transplantation for systemic disease had significantly lower rates of rejection. Four patients with sarcoidosis and 2 with lymphangioleiomyomatosis demonstrated recurrence in the allograft. Survival was similar between patients who underwent transplantation for systemic versus isolated disease.
  
  
  CONCLUSIONS
  Patients with respiratory failure resulting from these systemic diseases can undergo transplantation with outcomes comparable to those obtained in patients who undergo transplantation for isolated pulmonary disease.
authors.affiliation: Division of Cardiothoracic Surgery, Presbyterian University Hospital, University of Pittsburgh Medical Center, Pennsylvania 15213, USA.
authors.forename: F A
authors.initials: FA
authors.lastname: Pigula
authors.forename: B P
authors.initials: BP
authors.lastname: Griffith
authors.forename: M A
authors.initials: MA
authors.lastname: Zenati
authors.forename: J H
authors.initials: JH
authors.lastname: Dauber
authors.forename: S A
authors.initials: SA
authors.lastname: Yousem
authors.forename: R J
authors.initials: RJ
authors.lastname: Keenan
mesh_terms: D000328:Adult; D005260:Female; D006084:Graft Rejection; D006086:Graft vs Host Disease / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications; D007165:Immunosuppression Therapy / Q000379:methods; D007239:Infections / Q000209:etiology; D008168:Lung / Q000503:physiopathology; D008175:Lung Neoplasms / Q000150:complications; D016040:Lung Transplantation / Q000401:mortality; D008203:Lymphangiomyoma / Q000150:complications; D008297:Male; D011182:Postoperative Care / Q000379:methods; D011183:Postoperative Complications; D012008:Recurrence; D012131:Respiratory Insufficiency / Q000209:etiology / Q000601:surgery*; D012189:Retrospective Studies; D017565:Sarcoidosis, Pulmonary / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications