Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a defined clinicopathologic entity and has led to a significant increase in cases being diagnosed as both idiopathic PPFE or PPFE due to secondary causes, however the etiopathogenesis is unknown.
We studied the pathological features of lung lesions in 10 cases of idiopathic PPFE and 7 cases of bone marrow transplantation(BMT)-related cases. We also evaluated the elastic fiber related conditions of them with comparing to those of 7 cases of IIPF/UIP.
All lung lesions of idiopathic PPFE cases showed collapsed and marked elastofibrotic lesions in subpleural and peri-bronchovascular alveolar area associated with early fibrosis lesions, lymphangiogenesis, and infiltration of lymphocytes and eosinocytes in upper lobes. Lung lesions of 7 cases related to BMT had similar elastofibrotic lesions, but with bronchiolitis obliterans findings, and seen in the lower lobes in addition in upper lobes. Lung lesions of 3 cases of them have seen mainly in peri-bronchovascular alveolar area with organizing pneumonia findings instead of subpleural alveolar area.
In the all lesions of 17 PPEF cases, elastic fibers in the alveolar septal walls remained and newly formed elastic fibers were seen around the early fibrotic lesions with latent TGFβ-binding protein2 (LTBP2) expression.
In the lesions of UIP cases, elastic fibers in the alveolar septa tended to disappear and early fibrotic lesions were seen without findings of newly formed elastic fiber nor LTBP2 expression.
The elastic fiber related conditions around early fibrosis lesions may be different between two lesions.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, OA247.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019
