Idiopathic Pleuroparenchymale Fibroelastosis

Idiopathic pleuroparenchymale fibroelastosis is a rare, idiopathic interstitial pneumonia, which affects mainly the upper lung lobes and is slowly progressive. The patients often have recurrent infections, shortness of breath and dry cough. A diagnosis is made with high-resolution CT corticosteroids can be administered.

Idiopathic pleuroparenchymale fibroelastosis (ppfe) is a rare disease, which is classified as idiopathic interstitial pneumonia (1). Upper lobe fibrosis of the pleura and subpleural lung parenchyma included.

Idiopathic pleuroparenchymale fibroelastosis is a rare, idiopathic interstitial pneumonia, which affects mainly the upper lung lobes and is slowly progressive. The patients often have recurrent infections, shortness of breath and dry cough. A diagnosis is made with high-resolution CT corticosteroids can be administered. Idiopathic pleuroparenchymale fibroelastosis (ppfe) is a rare disease, which is classified as idiopathic interstitial pneumonia (1). Upper lobe fibrosis of the pleura and subpleural lung parenchyma included. Note 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 is unknown, but clinical data suggest an association with recurrent lung infection. It is also believed that playing genetic and autoimmune mechanisms play a role in this disease. Symptoms and signs The mean age of onset is at about 57 years without sexual preferences. Most patients are Non smoking. Patients report a history of often recurrent infections, shortness of breath and dry cough. Pneumothorax is often in the course of the disease. Diagnosis High-resolution CT (HRCT) to confirm surgical lung biopsy include the X-ray findings upper lobes pleural thickening and subpleural regions. You may have existing evidence from other interstitial pneumonias, including pattern of usual interstitial pneumonia and non-specific interstitial pneumonia simultaneously. Patients also have areas of consolidation and bronchiectasis. The pathology is characterized by intra-alveolar fibrosis, with alveolar walls in these areas that show a prominent elastosis and dense fibrous thickening of the pleura. In some patients, a coexistent interstitial pneumonia is in the lower wings. Surgical lung biopsy is needed to confirm the diagnosis. Prognosis The clinical course in patients with ppfe tends to be progressive in the majority of patients. Progression of the disease may occur in 60% of patients on therapy corticosteroids The appropriate treatment for this condition is unknown. Most of the literature reports the use of corticosteroids.

Health Life Media Team

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