Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis

Abstract

Background: Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is characterized by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma, with advanced cases often complicated by progressive weight loss. Therefore, we hypothesised that nutritional status is associated with mortality in iPPFE. Methods: This retrospective study assesses nutritional status at the time of diagnosis and one year after diagnosis in 125 patients with iPPFE. Malnutrition-related risk was evaluated using the Geriatric Nutritional Risk Index (GNRI). Results: Of the 125 patients, 96 (76.8%) were at malnutrition-related risk at the time of diagnosis according to GNRI. Of these, 21 patients (16.8%) were classified as at major malnutrition-related risk (GNRI <82). Patients at major malnutrition-related risk were significantly older and had worse pulmonary function than patients at low (92≤ GNRI <98)- and moderate (82≤ GNRI <92)-malnutrition-related risk. GNRI scores decreased significantly from the time of diagnosis to one year after diagnosis. Patients with lower GNRI (<91.7) had significantly shorter survival than patients with a median GNRI or higher (≥91.7). Patients with declines in annual GNRI scores of 5 or greater had significantly shorter survival than patients with declines in GNRI scores of less than 5. In multivariate analysis, major malnutrition-related risk was significantly associated with increased mortality after adjustment for age, sex and forced vital capacity (hazard-ratio, 1.946). A composite scoring model including age, sex and major malnutrition-related risk was able to separate mortality risk in iPPFE. Conclusion: Assessment of nutritional status by GNRI provides useful information for managing patients with iPPFE by predicting mortality risk. (250 words)

CT assessment of the normal upper-lobe lung volume in idiopathic pleuroparenchymal fibroelastosis

“…Notably, in addition to decreased normal LL volume, patients with iPPFE and lower-lobe ILD exhibited signi cantly decreased normal UL volume. In clinical practice, certain patients with iPPFE develop lower-lobe ILD during the course of the disease and the presence of lower-lobe ILD was reported to be associated with the risk of mortality [6][7][8][9]15]. Thus, the rate of advanced disease might have been higher in patients in the Discovery cohort.…”

“…Using age, sex and the normal UL volume, we developed a composite model that classi ed patients with iPPFE into three groups according to their mortality risk in the Combined cohort as well as the two separate cohorts. To date, several reports have proposed composite models [6][7][8][9]. The sensitivity and speci city of this model for predicting mortality risk were higher than those of the GAP index.…”

“…iPPFE was diagnosed using previously reported criteria [16]. First, the PPFE radiographic pattern on chest CT was de ned as bilateral subpleural dense consolidation with or without pleural thickening in the upper lobes and less marked or absent lower lobe involvement based on Reddy's radiological criteria [7]. Dense subpleural consolidation was observed below a line that was 1 cm from the lung's apex, with a minimum width of 1 cm in contact with the pleura.…”