(See also Overview of Idiopathic Interstitial Pneumonias Overview of Idiopathic Interstitial Pneumonias Idiopathic interstitial pneumonias are interstitial lung diseases that have no known cause that have some similarities in symptoms and how they affect the lungs. Some types of these diseases... read more .)
Idiopathic pleuroparenchymal fibroelastosis is a rare condition that is classified as an idiopathic interstitial pneumonia.
Idiopathic means the cause is unknown, but experts think the disorder may be related to recurrent lung infections. However, genetic and autoimmune factors may also play a role in idiopathic pleuroparenchymal fibroelastosis.
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 Pneumothorax A pneumothorax is the presence of air between the two layers of pleura (thin, transparent, two-layered membrane that covers the lungs and also lines the inside of the chest wall), resulting... read more 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.