title: Progression of fibrosis in usual interstitial pneumonia: serial evaluation of the native lung after single lung transplantation.
abstract: BACKGROUND
  Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a poor prognosis. Usual interstitial pneumonia (UIP) is the histopathological pattern identifying patients with the clinical entity of IPF. Despite aggressive immunosuppressive therapy the clinical course is usually dismal. For selected patients only lung transplantation improves prognosis and quality of life. After lung transplantation patients often receive a potent cyclosporine-based immunosuppressive therapy. Some reports suggest that cyclosporine has the potential to prevent progression of fibrosis.
  
  
  OBJECTIVE
  In patients with single lung transplantation (sLTx) for UIP we evaluated the effect of cyclosporine-based immunosuppressive therapy on progression of fibrosis using a high-resolution computed tomography (HRCT) scoring system.
  
  
  METHODS
  This retrospective observational study included 13 patients (24-64 years old) with histologically confirmed UIP who had HRCT scans preceding and following sLTx and who survived at least 6 months after sLTx. All patients were initially treated with cyclosporin A, prednisone and azathioprine. Three radiologists analyzed HRCT scans by setting a score regarding fibrosis [fibrosis score (FS); range 0-5 for each lobe] and ground-glass opacity [ground-glass score (GGS); range 0-5 for each lobe]. A comparison of serial changes (interval: 12-96 months posttransplant, 2-4 HRCT examinations/patient) was performed with the sign test.
  
  
  RESULTS
  Mean pretransplant FS and GGS of the nontransplanted lung were 1.80 and 1.61, respectively. Comparing pre- and posttransplant HRCT scans, mean lung FS significantly increased (0.35 +/- 0.15/year; p = 0.00024), while GGS tended to decrease (0.06 +/- 0.26/year; p = 0.5).
  
  
  CONCLUSIONS
  A cyclosporin A based triple immunosuppressive regimen following sLTx does not seem to prevent progression of the fibrotic changes of the native lung in patients with IPF.
authors.affiliation: Klinik für Nuklearmedizin, Universitätsklinikum des Saarlandes, Homburg, Saar, Deutschland. raagrg@uks.eu
authors.forename: Aleksandar
authors.initials: A
authors.lastname: Grgic
authors.forename: Henning
authors.initials: H
authors.lastname: Lausberg
authors.forename: Marc
authors.initials: M
authors.lastname: Heinrich
authors.forename: Jochem
authors.initials: J
authors.lastname: Koenig
authors.forename: Michael
authors.initials: M
authors.lastname: Uder
authors.forename: Gerhard W
authors.initials: GW
authors.lastname: Sybrecht
authors.forename: Heinrike
authors.initials: H
authors.lastname: Wilkens
mesh_terms: D000328:Adult; D016572:Cyclosporine / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use*; D008168:Lung / Q000000981:diagnostic imaging / Q000187:drug effects; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011014:Pneumonia / Q000150:complications*; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine