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Idiopathic Pleuroparenchymal Fibroelastosis

By

Joyce Lee

, MD, MAS, University of Colorado School of Medicine

Last full review/revision Jun 2021| Content last modified Jun 2021
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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.

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 Pneumothorax is common during the course of the disease.

Diagnosis

  • High-resolution CT (HRCT)

  • For confirmation, surgical lung biopsy

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.

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