Early intervention of nutritional education for idiopathic pleuroparenchymal fibroelastosis of interstitial lung disease

Abstract

Background

Lower body mass index (BMI) and weight loss are associated with disease progression and poor prognosis in patients with interstitial lung diseases (ILDs). Particularly in typically slim patients with idiopathic pleuroparenchymal fibroelastosis (iPPFE), prompt nutritional support may be essential.

Methods

We screened 93 consecutive patients diagnosed with PPFE at our hospital and included 63 patients with idiopathic PPFE in the final analysis. They were categorized into two groups—early intervention and non-early intervention—based on whether they received nutritional education by dietitian nutritionists within 6 months of diagnosis. We assessed risk factors for mortality using Cox regression analysis and evaluated body weight trajectories over time using individual scatter plots with regression lines.

Results

Among the 63 patients, 22 received early intervention and 41 did not. Early intervention in patients at an initial stage—defined in this study as FVC ≥70 % and no significant weight loss in the past year—was associated with a significantly slower rate of body weight decline compared to non-early intervention. However, survival did not differ significantly between groups regardless of FVC or weight loss status at diagnosis.

Conclusions

We outlined a structured nutritional education program delivered by dietitian nutritionists at our hospital. While the intervention did not improve survival, early nutritional education in patients at an initial stage slowed the rate of weight loss, which may contribute to improved prognosis. Maintaining body weight through early nutritional support may be an important therapeutic strategy in the management of iPPFE.