Idiopathic pleuroparenchymal fibroelastosis
Synonyms: IPPFE | Idiopathic pleuropulmonary fibroelastosis
A rare idiopathic interstitial pneumonia characterized by prominent subpleural and parenchymal fibroelastosis and pleural fibrosis predominantly involving the upper lobes. Signs and symptoms include non-productive cough dyspnea and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis architectural distortion and loss of volume) and reticulation.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version October 2023.
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Idiopathic pleuroparenchymal fibroelastosis?
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