Abstract
Patients with pleuroparenchymal fibroelastosis (PPFE) have severe breathlessness even with mild hypoxaemia. In patients with PPFE, accessory respiratory muscles, such as the sternocleidomastoid muscles, are used to maintain ventilation. An intense 18F‐fluorodeoxyglucose uptake in accessory respiratory muscles using positron emission tomography/computed tomography reflects the strong respiratory effort of patients with PPFE.
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