Abstract
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 thoracic dimension. Decreases in forced vital capacity, total lung capacity, and diffusing capacity are respiratory-function characteristics of PPFE, similar to those seen in idiopathic pulmonary fibrosis (IPF). The most remarkable difference in clinical features between PPFE and IPF is imaging findings, with upper-lobe-predominant lesions in PPFE and lower-lobe-predominant lesions in IPF.
Keywords: Pleuroparenchymal fibroelastosis (PPFE), pulmonary upper lobe fibrosis, pulmonary fibrosis (IPF).
Current Respiratory Medicine Reviews
Title:Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics
Volume: 9 Issue: 4
Author(s): Kentaro Watanabe
Affiliation:
Keywords: Pleuroparenchymal fibroelastosis (PPFE), pulmonary upper lobe fibrosis, pulmonary fibrosis (IPF).
Abstract: 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 thoracic dimension. Decreases in forced vital capacity, total lung capacity, and diffusing capacity are respiratory-function characteristics of PPFE, similar to those seen in idiopathic pulmonary fibrosis (IPF). The most remarkable difference in clinical features between PPFE and IPF is imaging findings, with upper-lobe-predominant lesions in PPFE and lower-lobe-predominant lesions in IPF.
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Watanabe Kentaro, Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics, Current Respiratory Medicine Reviews 2013; 9 (4) . https://dx.doi.org/10.2174/1573398X0904140129125307
DOI https://dx.doi.org/10.2174/1573398X0904140129125307 |
Print ISSN 1573-398X |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6387 |
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