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. Glucocorticoids may be given. Prognosis is poor.
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. Precise prevalence data are lacking.
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 (1). Genetic and autoimmune mechanisms are thought to play a role in this disease.
Etiology reference
1. Chua F, Desai SR, Nicholson AG, et al. Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics. Ann Am Thorac Soc 2019;16(11):1351-1359. doi:10.1513/AnnalsATS.201902-181CME
Symptoms and Signs of Idiopathic PPFE
The median age of presentation is approximately 49 years, with no clear sex predilection. Patients often report a history of recurrent infections, shortness of breath, and dry cough. Pneumothorax is common during the course of the disease. Anteroposterior shortening of the thorax (platythorax) is possible due to progressive fibrosis, especially affecting the upper lobes of the lungs. Undernutrition is common in patients with advanced 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 also have areas of consolidation and bronchiectasis. Subpleural cysts and thickening may be present.
On histological examination, patients can have co-existing findings of other interstitial pneumonias, including usual interstitial pneumonia and nonspecific interstitial pneumonia pattern.
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 with advanced disease, 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 glucocorticoids
Antifibrotic therapy
The optimal treatment for idiopathic pleuroparenchymal fibroelastosis is unknown (1). The majority of the literature reports the use of glucocorticoids.
In patients with significant morbidity, antifibrotics such as nintedanib and pirfenidone that are used for idiopathic pulmonary fibrosis or progressive fibrotic interstitial lung diseases may help slow the decline of lung function. As the disease is progressive, surgical resection of fibrotic foci or In patients with significant morbidity, antifibrotics such as nintedanib and pirfenidone that are used for idiopathic pulmonary fibrosis or progressive fibrotic interstitial lung diseases may help slow the decline of lung function. As the disease is progressive, surgical resection of fibrotic foci orlung transplantation may be considered in advanced cases (where hypercapnia and impending respiratory failure are present).
Treatment reference
1. Suzuki Y. Idiopathic pleuroparenchymal fibroelastosis: A review of the previous literature and current knowledge. Respir Investig 2025;63(1):127-137. doi:10.1016/j.resinv.2024.12.009
Prognosis for Idiopathic PPFE
In the majority of patients, the clinical course of idiopathic pleuroparenchymal fibroelastosis tends to be progressive. Disease progression occurs in most patients; 5-year survival rates are 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
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