Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics
نویسندگان
چکیده
منابع مشابه
Pleuroparenchymal Fibroelastosis: Its Pathological Characteristics
Pleuroparenchymal fibroelastosis (PPFE) is a distinct pattern of pulmonary fibrosis which often runs a rapidly progressive course with a poor prognosis, and it is likely to be introduced as a separate entity in the new classification scheme of idiopathic interstitial pneumonias. It is characterised by pleural fibrosis and subpleural fibroelastosis, with an upper lobe predominance. In addition t...
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...
متن کاملIdiopathic Pleuroparenchymal Fibroelastosis
PURPOSE OF THE REVIEW Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare fibrosing lung disease, affecting the visceral pleura and the subpleural parenchyma with an upper lobe predilection, included as a distinct clinicopathologic entity in the latest international multidisciplinary classification of the idiopathic interstitial pneumonias (IIP). We aim to summarize the current eviden...
متن کاملHistological evolution of pleuroparenchymal fibroelastosis
AIMS To investigate the histological evolution in the development of pleuroparenchymal fibroelastosis (PPFE). METHODS AND RESULTS We examined four patients who had undergone surgical lung biopsy twice, or who had undergone surgical lung biopsy and had been autopsied, and in whom the histological diagnosis of the first biopsy was not PPFE, but the diagnosis of the second biopsy or of the autop...
متن کاملPleuroparenchymal fibroelastosis: a rare interstitial lung disease
Pleuroparenchymal fibroelastosis (PPFE) is a newly described form of interstitial lung disease that originates in the upper lung zones and typically progresses to involve the entire lung. The disease may be idiopathic but is often associated with other pre- or coexisting conditions. Pneumothorax is a common complication and can occur at presentation or at other times during the course of the di...
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ژورنال
عنوان ژورنال: Annals of the American Thoracic Society
سال: 2019
ISSN: 2329-6933,2325-6621
DOI: 10.1513/annalsats.201902-181cme