Upper lobe fibrosis: a novel manifestation of chronic allograft dysfunction in lung transplantation.

Published

Journal Article

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. CONCLUSION: 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.

Full Text

Duke Authors

Cited Authors

  • Pakhale, SS; Hadjiliadis, D; Howell, DN; Palmer, SM; Gutierrez, C; Waddell, TK; Chaparro, C; Davis, RD; Keshavjee, S; Hutcheon, MA; Singer, LG

Published Date

  • September 2005

Published In

Volume / Issue

  • 24 / 9

Start / End Page

  • 1260 - 1268

PubMed ID

  • 16143243

Pubmed Central ID

  • 16143243

Electronic International Standard Serial Number (EISSN)

  • 1557-3117

International Standard Serial Number (ISSN)

  • 1053-2498

Digital Object Identifier (DOI)

  • 10.1016/j.healun.2004.08.026

Language

  • eng