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.
Export Options
About this article
Cite this article as:
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 |
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
Stem Cell-Derived Microvesicles: A Cell Free Therapy Approach to the Regenerative Medicine
Current Biotechnology Adrenomedullin in Vascular Endothelial Injury and Combination Therapy: Time for a New Paradigm
Current Vascular Pharmacology Utilization of Microfluidics for the Preparation of Polymeric Nanoparticles for the Antioxidant Rutin: A Comparison with Bulk Production
Pharmaceutical Nanotechnology Cholesterol, Copper and Aβ in Controls, MCI, AD and the AD Cholesterol- Lowering Treatment Trial (ADCLT)
Current Alzheimer Research Matrix Metalloproteinases as Potential Targets in the Venous Dilation Associated with Varicose Veins
Current Drug Targets Progestins for Symptomatic Endometriosis: Results of Clinical Studies
Current Drug Therapy MicroRNAs in the Management of Heart Failure
Current Medicinal Chemistry Objectively Assessed Physical Activity and Sedentary Behaviour During Pregnancy in Portuguese Women: Differences Between Trimesters and Weekdays and Weekends
Current Women`s Health Reviews The Dynamics of the Hypothalamic-Pituitary-Ovarian Axis, ReproductivePerformance and Sexuality Following Bariatric Surgery
Current Women`s Health Reviews Clinical Aspects of Melatonin in the Acute Coronary Syndrome
Current Vascular Pharmacology Inflammation, Sleep, Obesity and Cardiovascular Disease.
Current Vascular Pharmacology Modulation of Neutrophil Function by Hormones
Current Immunology Reviews (Discontinued) Current and Experimental Antibody-Based Therapeutics: Insights, Breakthroughs, Setbacks and Future Directions
Current Molecular Medicine Anti-Inflammatory Agents in Mouth-Rinses For Periodontal Treatment
Clinical Anti-Inflammatory & Anti-Allergy Drugs (Discontinued) Is Red Meat Required for the Prevention of Iron Deficiency Among Children and Adolescents?
Current Pediatric Reviews Hypoxia in Pre-Eclampsia: Cause or Effect?
Current Women`s Health Reviews Global Trends of Computational Fluid Dynamics to Resolve Real World Problems in the Contemporary Era
Current Biochemical Engineering (Discontinued) Multifunctional Anti-Cancer Nano-Platforms are Moving to Clinical Trials
Current Drug Metabolism Type 2 Diabetes Mellitus and the Elderly: An Update on Drugs Used to Treat Glycaemia
Current Vascular Pharmacology Patent Selections
Recent Patents on Cardiovascular Drug Discovery