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 idiopathic interstitial pneumonia (1). 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 of Idiopathic PPFE
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 of Idiopathic PPFE
The median age of presentation is around 57 years, with no sex predilection. Most patients with idiopathic pleuroparenchymal fibroelastosis do not smoke. Patients often report a history of recurrent infections, shortness of breath, and dry cough. Pneumothorax is common during the course of the disease.
Diagnosis of Idiopathic PPFE
High-resolution CT (HRCT)
For confirmation, 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 and nonspecific interstitial pneumonia pattern. Patients can also have areas of consolidation and bronchiectasis.
Image courtesy of Joyce S. Lee, MD, MAS.
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.
Treatment of Idiopathic PPFE
Possibly corticosteroids
The appropriate treatment for idiopathic pleuroparenchymal fibroelastosis is unknown. The majority of the literature reports the use of corticosteroids.
Prognosis for Idiopathic PPFE
The clinical course in patients with idiopathic pleuroparenchymal fibroelastosis tends to be progressive in the majority of patients. Disease progression occurs in most patients with 5 year survival rates approximately 25 to 60% (1).
Prognosis reference
1. Kinoshita Y, Ikeda T, Miyamura T, et al. A proposed prognostic prediction score for pleuroparenchymal fibroelastosis. Respir Res 22: 215, 2021. https://doi.org/10.1186/s12931-021-01810-z
