Clinical features of idiopathic pleuroparenchymal fibroelastosis with progressive phenotype showing a decline in forced vital capacity

Kono, Masato; Tsunoda, Tomo; Ikeda, S.; Yagi, Shota; Hirama, Ryutaro; Watanuki, Masayuki; Oshima, Yuiko; Tsutsumi, Akari; Miwa, Hideki; Miki, Yuko; Dai, Huaping; Suda, Takafumi; Nakamura, Hidenori

doi:10.1016/j.resinv.2023.01.003

Cited by 3 publications

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“…Indeed, our previous study reported that muscle wasting is frequently observed and body composition change evaluated by elector spine muscle attenuation on CT is associated with mortality in patients with iPPFE [26]. In line with our results, interestingly, body-weight loss was strongly correlated with FVC decline in patients with iPPFE and IPF [29,30]. These results suggest an underlying mechanism between the pathogenesis of lung fibrosis and deterioration of nutritional status.…”

Section: Discussionsupporting

confidence: 91%

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

et al. 2023

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BackgroundIdiopathic pleuroparenchymal fibroelastosis (iPPFE) is characterised by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma, with advanced cases often complicated by progressive weight loss. Therefore, we hypothesized that nutritional status is associated with mortality in iPPFE.MethodsThis retrospective study assesses nutritional status at the time of diagnosis and one year after diagnosis in 131 patients with iPPFE. Malnutrition-related risk was evaluated using the Geriatric Nutritional Risk Index (GNRI).ResultsOf the 131 patients, 96 (76.3%) were at malnutrition-related risk at the time of diagnosis according to GNRI. Of these, 21 patients (16.0%) 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 (≥91.8). 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, 1.957). A composite scoring model including age, sex, and major malnutrition-related risk was able to separate mortality risk in iPPFE.ConclusionAssessment of nutritional status by GNRI provides useful information for managing patients with iPPFE by predicting mortality risk.

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Section: Discussionsupporting

confidence: 91%