PT  - JOURNAL ARTICLE
AU  - Laurent Plantier
AU  - Aurélie Cazes
AU  - Anh-Tuan Dinh-Xuan
AU  - Catherine Bancal
AU  - Sylvain Marchand-Adam
AU  - Bruno Crestani
TI  - Physiology of the lung in idiopathic pulmonary fibrosis
AID  - 10.1183/16000617.0062-2017
DP  - 2018 Mar 31
TA  - European Respiratory Review
PG  - 170062
VI  - 27
IP  - 147
4099  - http://err.ersjournals.com/content/27/147/170062.short
4100  - http://err.ersjournals.com/content/27/147/170062.full
SO  - EUROPEAN RESPIRATORY REVIEW2018 Mar 31; 27
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