Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics
Open Access
- 31 January 2014
- journal article
- Published by Bentham Science Publishers Ltd. in Current Respiratory Medicine Reviews
- Vol. 9 (4), 229-237
- https://doi.org/10.2174/1573398X0904140129125307
Abstract
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
This publication has 30 references indexed in Scilit:
- An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial PneumoniasAmerican Journal of Respiratory and Critical Care Medicine, 2013
- Idiopathic Pleuroparenchymal FibroelastosisSocial psychiatry. Sozialpsychiatrie. Psychiatrie sociale, 2004
- [Idiopathic pulmonary fibrosis of the upper lobe: a case report].1999
- [Idiopathic progressive pleuropulmonary fibrosis. Apropos of 2 cases].1984
- Pulmonary apical fibrocystic disease. A serologic study.1981
- HLA-antigens and pulmonary upper lobe fibrocystic changes with and without ankylosing spondylitis. A report of seven cases.1978
- Idiopathic progressive pulmonary fibrosis.Thorax, 1975
- Ankylosing spondylitis and lung fibrosis.1972
- A case of idiopathic cavitation of lung demonstrated at the Postgraduate Medical School of London.BMJ, 1966
- Undiagnosable Lung Disease.1962