The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis.
نویسندگان
چکیده
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منابع مشابه
Two Different Aspects of Pleuroparenchymal Fibroelastosis: A Disease of Pulmonary Fibrosis, and of the Chest Wall
The essential histology of PPFE is subpleural fibroelastosis in the upper lobes, which is different from that of usual interstitial pneumonia (UIP). However, PPFE might share a pathologic process leading to end-stage fibrosis with UIP: it might have an antecedent inflammatory or acute lung injury process prior to the development of the thick subleural bands of fibroelastosis, possibly correspon...
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References 1 Frankel SK, Cool CD, Lynch DA, et al. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004; 126: 2007–2013. 2 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...
متن کاملPleuroparenchymal Fibroelastosis: Its Clinical Characteristics
Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upperlobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough. Chest pain because of pneumothorax may be the first symptom in some patients. Patients with PPFE are slender with a flat rib cage or abnormally narrowed anterior-posterior tho...
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RATIONAL Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease that is characterized radiologically by apical pleural thickening and histologically by elastic fibrosis of the visceral pleura. Although PPFE cases have been reported occasionally since this disease was initially described, most such cases have involved secondary PPFE. Idiopathic PPFE (iPPFE) cases have been l...
متن کاملIdiopathic pleuroparenchymal fibroelastosis – A rare idiopathic interstitial pneumonia
Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia. It was first described in 2004 and subsequently included in the ATS/ERS classification of idiopathic interstitial pneumonia in 2013. There have been few cases reported so far. The diagnostic criteria is still emerging and its etiology is being questioned. We report a case of pleuroparenchymal fibroelastosis...
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ورودعنوان ژورنال:
- European respiratory review : an official journal of the European Respiratory Society
دوره 23 132 شماره
صفحات -
تاریخ انتشار 2014