Physiology of the lung in idiopathic pulmonary fibrosis

Eur Respir Rev. 2018 Jan 24;27(147):170062. doi: 10.1183/16000617.0062-2017. Print 2018 Mar 31.

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.

Publication types

  • Review

MeSH terms

  • Animals
  • Cough / physiopathology
  • Hemodynamics
  • Humans
  • Hypoxia / physiopathology
  • Idiopathic Pulmonary Fibrosis / pathology
  • Idiopathic Pulmonary Fibrosis / physiopathology*
  • Lung / pathology
  • Lung / physiopathology*
  • Lung Compliance
  • Pulmonary Circulation
  • Pulmonary Diffusing Capacity
  • Pulmonary Ventilation*
  • Respiratory Dead Space
  • Respiratory Mechanics*