Diffuse Alveolar Damage Is Not a Prominent Feature of Non-Lung-Transplant-Related Pleuroparenchymal Fibroelastosis
Jeffrey J Tanguay, Efrat Ofek, Shaf Keshavjee, Thomas K Waddell, Marcio M Gomes, David M Hwang. University Health Network, Toronto, ON, Canada; Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada
Background: Pleuroparenchymal fibroelastosis (PPFE) is a clinicopathological entity characterized by pleural and subpleural upper lobe predominate fibrosis and fibroelastosis in patients presenting with a chronic interstitial pneumonia. The etiology and pathogenesis of PPFE remain poorly understood. PPFE was recently described as a major histologic correlate of post-lung transplant restrictive allograft syndrome, and has been described following bone marrow transplantation (BMT). We reviewed our files to identify cases of non-lung-transplant-related PPFE and assess histopathologic features and clinical associations.
Design: Lung biopsy and resection cases from 2002 to 2012 containing areas of fibroelastosis, infarct-like fibrosis, or apical cap-like fibrosis were identified and reviewed. Histopathological findings were assessed by three pathologists with pulmonary expertise. Cases of restrictive allograft syndrome were excluded.
Results: In total, 37 cases were identified, of which 12 demonstrated typical features of PPFE. Of these, 5 were idiopathic and 7 were in post-BMT patients. Most cases showed subpleural, paraseptal, and peribronchovascular distribution of fibroelastosis. Diffuse alveolar damage (DAD) was identified in only 1 case. All idiopathic cases occurred in females aged 20 to 64. The post-BMT group included 6 females and 1 male, aged 17 to 52, with BMT occurring 3 to 13 years prior to the pathology specimen. Four of 7 post-BMT patients developed clinical graft versus host disease (GVHD), while two additional patients showed histologic features suggestive of GVHD.
Conclusions: While some cases are idiopathic, PPFE may also develop as a late complication of BMT. In contrast to PPFE in post-lung transplant restrictive allograft syndrome, diffuse alveolar damage is not a prominent feature in either idiopathic or post-BMT cases of PPFE.
Tuesday, March 5, 2013 1:00 PM
Poster Session IV # 291, Tuesday Afternoon