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.
Abstract
Physiological impairment in IPF is complex and involves all compartments of the respiratory system http://ow.ly/gyao30hdHUb
Footnotes
Conflict of interest: None declared.
Provenance: Submitted article, peer reviewed.
- Received May 22, 2017.
- Accepted October 15, 2017.
- Copyright ©ERS 2018.
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