Pulmonary function testing in patients with neuromuscular disease

Abstract

Progressive neuromuscular disease leads to muscle weakness or failure that produces loss of pulmonary function and clinical respiratory morbidity. Tracking pulmonary function in a practical and effective way is very important because it can help identify a stage of disease when a morbidity, such as inadequate airway clearance or respiratory failure, may be present. There are four general categories of pulmonary function outcome measures such as volume, flow, pressure, and gas exchange. These outcome measures have variable precision and accuracy in predicting clinical change, and practicality in performing them relative to age and condition. It is widely recommended to follow multiple measurements longitudinally and create an accurate and timely clinical picture. This manuscript will review the most commonly used and most practical measures for use in clinical practice and how they can help to assess morbidity, disease state, and help optimize patient management.