Exertional dyspnoea in interstitial lung diseases: the clinical utility of cardiopulmonary exercise testing

Abstract

Interstitial lung diseases (ILDs) represent a heterogeneous group of pathologies characterised by alveolar and interstitial damage, pulmonary inflammation (usually associated with fibrosis), decreased lung function and impaired gas exchange, which can be attributed to either a known or an unknown aetiology. Dyspnoea is one of the most common and disabling symptoms in patients with ILD, significantly impacting quality of life. The mechanisms causing dyspnoea are complex and not yet fully understood. However, it is recognised that dyspnoea occurs when there is an imbalance between the central respiratory efferent drive and the response of the respiratory musculature. The respiratory derangement observed in ILD patients at rest is even more evident during exercise. Pathophysiological mechanisms responsible for exertional dyspnoea and reduced exercise tolerance include altered respiratory mechanics, impaired gas exchange, cardiovascular abnormalities and peripheral muscle dysfunction.This review describes the respiratory physiology of ILD, both at rest and during exercise, and aims to provide comprehensive and updated evidence on the clinical utility of the cardiopulmonary exercise test in the assessment and management of these pathological entities. In addition, the role of exercise training and pulmonary rehabilitation programmes in the ILD population is addressed.

FIGURE 1

Idiopathic and non-idiopathic interstitial lung disease (ILD). IIP: idiopathic interstitial pneumonia; CTD: connective tissue disease; IPAF: interstitial pneumonia with autoimmune features; LIP: lymphocytic interstitial pneumonia; PPFE: pleuroparenchymal fibroelastosis; COP: cryptogenic organising pneumonia; AIP: acute interstitial pneumonitis; IPF: idiopathic pulmonary fibrosis; NSIP: nonspecific interstitial pneumonia; RB: respiratory bronchiolitis; DIP: desquamative interstitial pneumonia.