Affiliation: Department of Pathology, Toronto General Hospital, Room 11th Floor, 200 Elizabeth Street, Toronto, Ontario, M5G 2C4, Canada.
Pleuroparenchymal fibroelastosis (PPFE) is a clinicopathologic entity characterized by clinical presentation suggestive of a chronic idiopathic interstitial pneumonia, radiologic features of pleural and parenchymal involvement accentuated in the upper lobes, and a constellation of histologic findings including visceral pleural fibrosis and prominent fibroelastosis of the subpleural lung parenchyma. While the large majority of cases of PPFE have been considered idiopathic, development of PPFE in post-bone marrow transplant patients and in lung transplant recipients has recently been reported. Further, with the growing number of idiopathic PPFE cases reported in the literature, interesting patterns of association between PPFE and various clinical conditions – including prior treatment for malignancy, autoimmunity, recurrent infections, and vascular compromise/ischemia – are beginning to emerge. These associations, reviewed here, may offer clues into the pathogenesis of this rare condition.