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Idiopathic Pleuroparenchymal Fibroelastosis

By Joyce Lee, MD, MAS, Assistant Professor, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Denver

Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia that is slowly progressive.

  • People often have recurrent infections, shortness of breath, and a dry cough.

  • Doctors use computed tomography and lung biopsy to diagnose the disease.

  • Treatment usually involves giving corticosteroids.

Idiopathic pleuroparenchymal fibroelastosis is a rare condition that is classified as an idiopathic interstitial pneumonia.

The cause is unknown, but experts think it may be related to recurrent lung infections. However, genetic and autoimmune factors may also play a role in this disease.

Men and women are affected about evenly. Idiopathic pleuroparenchymal fibroelastosis occurs in people of all ages. However, the median age when the disease starts is around 57 years. Most affected people are nonsmokers.

People may have recurrent infections, shortness of breath, and a dry cough. Pneumothorax is common during the course of the disease.

Diagnosis of idiopathic pleuroparenchymal fibroelastosis requires chest computed tomography (CT). Sometimes a biopsy of the lung is needed to confirm the diagnosis.

The appropriate treatment for this condition is unknown. Sometimes doctors give corticosteroids because they are effective in some of the other idiopathic interstitial pneumonias, but their effectiveness in idiopathic pleuroparenchymal fibroelastosis is unknown.