- Overview of Interstitial Lung Diseases
- Overview of Idiopathic Interstitial Pneumonias
- Acute Interstitial Pneumonia
- Cryptogenic Organizing Pneumonia
- Idiopathic Pleuroparenchymal Fibroelastosis
- Idiopathic Pulmonary Fibrosis
- Lymphoid Interstitial Pneumonia
- Nonspecific Interstitial Pneumonia
- Respiratory Bronchiolitis–Associated Interstitial Lung Disease and Desquamative Interstitial Pneumonia
- Drug-Induced Pulmonary Disease
- Eosinophilic Pneumonia
- Hypersensitivity Pneumonitis
- Lymphangioleiomyomatosis
- Pulmonary Alveolar Proteinosis
- Pulmonary Langerhans Cell Histiocytosis
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 sometimes lung biopsy to diagnose the disease.
Treatment usually involves giving steroids.
(See also Overview of Idiopathic Interstitial Pneumonias.)
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 do not smoke.
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 steroids (sometimes called glucocorticoids or corticosteroids) because they are effective in some of the other idiopathic interstitial pneumonias, but their effectiveness in idiopathic pleuroparenchymal fibroelastosis is unknown. Sometimes antifibrotic medications (for example, nintedanib or pirfenidone) are tried to slow the decline of lung function. Occasionally The appropriate treatment for this condition is unknown. Sometimes doctors give steroids (sometimes called glucocorticoids or corticosteroids) because they are effective in some of the other idiopathic interstitial pneumonias, but their effectiveness in idiopathic pleuroparenchymal fibroelastosis is unknown. Sometimes antifibrotic medications (for example, nintedanib or pirfenidone) are tried to slow the decline of lung function. Occasionallylung transplantation may be done.
The disease progresses in most people The 5-year survival rates range from 25 to 60%.
