Positive Effects of Nutritional Education in a Patient With Pleuroparenchymal Fibroelastosis

Pleuroparenchymal fibroelastosis (PPFE) patients often experience dyspnea, which can lead to weight loss due to increased calorie expenditure from the effort required for breathing. Weight loss in PPFE has been associated with a decline in lung function, which, in turn, may contribute to a poor prognosis. Therefore, nutritional therapy plays a critical role in managing this condition. Here, we report a case of a patient with idiopathic PPFE (iPPFE) who showed a clear positive response to nutritional therapy. The patient experienced a gradual decline in lung function (FVC) and body weight, leading to worsening respiratory symptoms. After being referred to our hospital, a chest CT confirmed the diagnosis of PPFE, and he began nutritional education focused on a high-fat, high-protein diet. Despite initial weight loss, his weight and FVC improved, and his respiratory symptoms eased. No pharmacological intervention was implemented throughout the course. The patient reported positive effects from nutritional therapy and remains under follow-up care. Our findings suggest that maintaining body weight and physique may help stabilize lung function and potentially slow the progression of PPFE.