Respiratory medicine case reports
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Respir Med Case Rep · Jan 2014
Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature.
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. ⋯ 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.