Cardiopulmonary exercise testing in interstitial lung diseases and the value of ventilatory efficiency

Interstitial lung diseases (ILDs) are diverse parenchymal pulmonary disorders, primarily characterised by alveolar and interstitial inflammation and/or fibrosis, and sharing pathophysiological similarities. Thus, patients generally harbour common respiratory symptoms, lung function abnormalities and modified exercise adaptation. The most usual and disabling complaint is exertional dyspnoea, frequently responsible for premature exercise interruption. Cardiopulmonary exercise testing (CPET) is increasingly used for the clinical assessment of patients with ILD. This is because exercise performance or dyspnoea on exertion cannot reliably be predicted by resting pulmonary function tests. CPET, therefore, provides an accurate evaluation of functional capacity on an individual basis. CPET can unmask anomalies in the integrated functions of the respiratory, cardiovascular, metabolic, peripheral muscle and neurosensory systems in ILDs. CPET uniquely provides an evaluation of all above aspects and can help clinicians shape ILD patient management. Preliminary evidence suggests that CPET may also generate valuable prognostic information in ILDs and can be used to shed light on the presence of associated pulmonary hypertension. This review aims to provide comprehensive and updated evidence concerning the clinical utility of CPET in ILD patients, with particular focus on the physiological and clinical value of ventilatory efficiency ( V ˙ E / V ˙ CO 2 ).