RT Journal Article
SR Electronic
T1 Physiology of the lung in idiopathic pulmonary fibrosis
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 170062
DO 10.1183/16000617.0062-2017
VO 27
IS 147
A1 Laurent Plantier
A1 Aurélie Cazes
A1 Anh-Tuan Dinh-Xuan
A1 Catherine Bancal
A1 Sylvain Marchand-Adam
A1 Bruno Crestani
YR 2018
UL http://err.ersjournals.com/content/27/147/170062.abstract
AB 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