pulmonary disorders
Idiopathic Pleuroparenchymal Fibroelastosis
Introduction
Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia that predominantly involves the upper lobes of the lungs and is slowly progressive. Patients often have recurrent infections, shortness of breath, and dry cough. Diagnosis is with high-resolution CT but sometimes requires lung biopsy. Corticosteroids may be given. Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare condition that is classified as an Overview of Idiopathic Interstitial Pneumonias ( 