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.
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.
Authors: Adriana Dubbeldam; Caroline Barthels; Johan Coolen; Johny A Verschakelen; Stijn E Verleden; Robin Vos; Geert M Verleden; Walter De Wever Journal: Eur Radiol Date: 2016-11-22 Impact factor: 5.315
Authors: Ariss DerHovanessian; Jamie L Todd; Alice Zhang; Ning Li; Aradhna Mayalall; C Ashley Finlen Copeland; Michael Shino; Elizabeth N Pavlisko; W Dean Wallace; Aric Gregson; David J Ross; Rajan Saggar; Joseph P Lynch; John Belperio; Laurie D Snyder; Scott M Palmer; S Sam Weigt Journal: Ann Am Thorac Soc Date: 2016-05
Authors: Emily F Fishman; James D Quirk; Stuart C Sweet; Jason C Woods; David S Gierada; Mark S Conradi; Marilyn J Siegel; Dmitriy A Yablonskiy Journal: Pediatr Transplant Date: 2017-01-24
Authors: Nevins W Todd; Sergei P Atamas; Stella E Hines; Irina G Luzina; Nirav G Shah; Edward J Britt; Andrew J Ghio; Jeffrey R Galvin Journal: Expert Rev Respir Med Date: 2022-01-31 Impact factor: 3.772