Abstract
Idiopathic Pleuroparenchymal Fibroelastosis (iPPFE) is a rare idiopathic interstitial pneumonia (IIP). It is characterized by dense collagenous fibrosis and/or elastosis of the visceral pleura and subpleural tissue. iPPFE predominantly originates in the upper lung zones and later leads to progression to involve the entire lung.
Etiology and pathophysiology still remains mystery for us. This disease has some typical signs which should be taken into consideration while thinking about possibility of having iPPFE.
It is typically a flat chest (platythorax) and in time developing hypercapnic respiratory insufficiency. Diagnosis is build up on clinical, radiological and histopathological findings.
Unfortunately, it is usually a progressive disease without knowing effective medication. Hence, lung transplantation is the only option for patient.