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We describe a patient with idiopathic pleuroparenchymal fibroelastosis (IPPFE). This rare clinicopathological syndrome is characterized by typical apical alterations op chest imaging, such as pleural thickening and subpleural fibrosis. Thickened visceral pleura and subpleural fibrosis consisting of dense collagen and elastin, are the main histopathological features. Etiology is unknown but a link between recurrent infections (in particular aspergillosis) and autoimmune diseases is suspected. At this time there is no standardized treatment regimen and the prognosis is variable.