Service de Pneumologie et Explorations Fonctionnelles Respiratoires, Hôpital Bretonneau, Tours, France.
Université François Rabelais, Tours, France.
Eur Respir Rev. 2018 Jan 24;27(147). doi: 10.1183/16000617.0062-2017. Print 2018 Mar 31.
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.
特发性肺纤维化 (IPF) 的临床表现与肺功能的多种改变直接相关。这些改变源自影响下呼吸道所有部位的复杂疾病过程，从传导气道到肺血管。在本文中，我们回顾了肺力学（肺顺应性和肺容积降低）、肺气体交换（弥散能力降低、死腔通气增加、慢性动脉低氧血症）和气道生理学（咳嗽反射增加、气道容积增加）以及与 IPF 相关的肺血流动力学的深刻改变。讨论了这些改变对 IPF 中运动受限和呼吸困难的相对贡献。
