Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare condition that is classified as an idiopathic interstitial pneumonia Overview of Idiopathic Interstitial Pneumonias Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic... read more (1 General reference Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic... read more ). It involves upper lobe fibrosis of the pleura and subpleural lung parenchyma.
General reference
1. Travis WD, Costabel U, Hansell DM, et al: An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 188 (6):733–748, 2013.
Etiology
The cause of idiopathic pleuroparenchymal fibroelastosis is unknown, but clinical data suggest a link to recurrent pulmonary infection. Genetic and autoimmune mechanisms are also thought to play a role in this disease.
Symptoms and Signs
The median age of presentation is around 57 years, with no sex predilection. Most patients with idiopathic pleuroparenchymal fibroelastosis are nonsmokers. Patients often report a history of recurrent infections, shortness of breath, and dry cough. Pneumothorax Pneumothorax Pneumothorax is air in the pleural space causing partial or complete lung collapse. Pneumothorax can occur spontaneously or result from trauma or medical procedures. Diagnosis is based on clinical... read more 
is common during the course of the disease.
Diagnosis
High-resolution CT (HRCT)
For confirmation, surgical lung biopsy
The imaging findings in idiopathic pleuroparenchymal fibroelastosis include upper lobe thickening of the pleura and subpleural regions. Patients can have co-existing findings of other interstitial pneumonias, including usual interstitial pneumonia Overview of Idiopathic Interstitial Pneumonias Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic... read more and nonspecific interstitial pneumonia pattern. Patients can also have areas of consolidation and bronchiectasis Bronchiectasis Bronchiectasis is dilation and destruction of larger bronchi caused by chronic infection and inflammation. Common causes are cystic fibrosis, immune defects, and recurrent infections, though... read more 
.
The pathology is characterized by intra-alveolar fibrosis with the alveolar walls in these areas showing prominent elastosis and dense fibrous thickening of the visceral pleura. In some patients, there is co-existent interstitial pneumonia in the lower lobes.
Surgical lung biopsy is required for confirmation of the diagnosis.
Prognosis
The clinical course in patients with idiopathic pleuroparenchymal fibroelastosis tends to be progressive in the majority of patients. Disease progression occurs in 60% of patients.
Treatment
Possibly corticosteroids
The appropriate treatment for idiopathic pleuroparenchymal fibroelastosis is unknown. The majority of the literature reports the use of corticosteroids.
