Physiology of the lung in idiopathic pulmonary fibrosis. (24th January 2018)

• Record Type: Journal Article
• Authors: Plantier, Laurent; Cazes, Aurélie; Dinh-Xuan, Anh-Tuan; Bancal, Catherine; Marchand-Adam, Sylvain; Crestani, Bruno
• Is Part Of: European respiratory review. Volume 27:Number 147(2018)
• Publication Date: 2018-01-24
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases; Respiratory organs -- Diseases; Respiratory organs -- Diseases -- Treatment; Electronic journals; Periodical; Periodicals; Periodicals; 616.2
• DOI: 10.1183/16000617.0062-2017
• Languages: English
• ISSNs: 0905-9180

Abstract

The clinical expression of idiopathic pulmonary fibrosis (IPF) is directly related to multiple alterations in lung function. These alterations derive from a complex disease process affecting all compartments of the lower respiratory system, from the conducting airways to the lung vasculature. In this article we review the profound alterations in lung mechanics (reduced lung compliance and lung volumes), pulmonary gas exchange (reduced diffusing capacity, increased dead space ventilation, chronic arterial hypoxaemia) and airway physiology (increased cough reflex and increased airway volume), as well as pulmonary haemodynamics related to IPF. The relative contribution of these alterations to exertional limitation and dyspnoea in IPF is discussed. Physiological impairment in IPF is complex and involves all compartments of the respiratory system http://ow.ly/gyao30hdHUb