title: Upper lobe fibrosis: a novel manifestation of chronic allograft dysfunction in lung transplantation.
abstract: BACKGROUND
  Lung transplantation is an established treatment modality for a number of chronic lung diseases. Long-term survival after lung transplantation is limited by chronic allograft dysfunction, usually manifested by bronchiolitis obliterans syndrome. We describe a case series with upper lobe fibrosis, a novel presentation of chronic allograft dysfunction.
  
  
  METHODS
  We reviewed lung transplants at the Toronto General Hospital and Duke University Hospital from 1990 to 2002 and identified patients with upper lobe fibrosis.
  
  
  RESULTS
  Thirteen of 686 patients (6 women) developed upper lobe fibrosis (Toronto, 9; Duke, 4); 12 of 13 had bilateral transplants. The median age at diagnosis was 42 years (range, 19-70). Primary diagnoses were cystic fibrosis, 6; emphysema, 4; sarcoidosis, 1; and pulmonary fibrosis, 2 patients. Radiographic diagnosis was made at a median of 700 days post-transplant (range, 150-2,920). Pulmonary function tests demonstrated predominantly a progressively worsening restrictive pattern. Open lung biopsy specimens revealed dense interstitial fibrosis, with occasional features of obliterative bronchitis, bronchiolitis obliterans obstructive pneumonia, and aspiration. Nine patients died at a median follow-up of 2,310 days (range, 266-3,740), 8 due to respiratory failure.
  
  
  CONCLUSIONS
  Upper lobe fibrosis is a novel presentation of chronic allograft dysfunction in lung transplant recipients and is differentiated from bronchiolitis obliterans syndrome on the basis of physiologic and radiologic findings.
authors.affiliation: Toronto Lung Transplant Program, University Health Network, Toronto, Ontario, Canada.
authors.forename: Smita Sakha
authors.initials: SS
authors.lastname: Pakhale
authors.forename: Denis
authors.initials: D
authors.lastname: Hadjiliadis
authors.forename: David N
authors.initials: DN
authors.lastname: Howell
authors.forename: Scott M
authors.initials: SM
authors.lastname: Palmer
authors.forename: Carlos
authors.initials: C
authors.lastname: Gutierrez
authors.forename: Thomas K
authors.initials: TK
authors.lastname: Waddell
authors.forename: Cecilia
authors.initials: C
authors.lastname: Chaparro
authors.forename: R Duane
authors.initials: RD
authors.lastname: Davis
authors.forename: Shaf
authors.initials: S
authors.lastname: Keshavjee
authors.forename: Michael A
authors.initials: MA
authors.lastname: Hutcheon
authors.forename: Lianne G
authors.initials: LG
authors.lastname: Singer
mesh_terms: D000328:Adult; D000368:Aged; D001228:Aspergillosis / Q000209:etiology; D001232:Aspergillus fumigatus; D018549:Cryptogenic Organizing Pneumonia / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016040:Lung Transplantation / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000473:pathology; D011552:Pseudomonas Infections / Q000209:etiology; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D011859:Radiography; D012189:Retrospective Studies