Upper lobe fibrosis: a novel manifestation of chronic allograft dysfunction in lung transplantation.
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.
Pakhale
Smita Sakha
SS
Toronto Lung Transplant Program, University Health Network, Toronto, Ontario, Canada.
Hadjiliadis
Denis
D
Howell
David N
DN
Palmer
Scott M
SM
Gutierrez
Carlos
C
Waddell
Thomas K
TK
Chaparro
Cecilia
C
Davis
R Duane
RD
Keshavjee
Shaf
S
Hutcheon
Michael A
MA
Singer
Lianne G
LG
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