RT Journal Article SR Electronic T1 Assessment of malnutrition-related risk in patients with idiopathic pleuroparenchymal fibroelastosis JF ERJ Open Research JO erjor FD European Respiratory Society SP 00749-2022 DO 10.1183/23120541.00749-2022 A1 Yuzo Suzuki A1 Atsuki Fukada A1 Kazutaka Mori A1 Masato Kono A1 Hirotsugu Hasegawa A1 Dai Hashimoto A1 Koshi Yokomura A1 Shiro Imokawa A1 Yusuke Inoue A1 Hideki Yasui A1 Hironao Hozumi A1 Masato Karayama A1 Kazuki Furuhashi A1 Noriyuki Enomoto A1 Tomoyuki Fujisawa A1 Naoki Inui A1 Hidenori Nakamura A1 Takafumi Suda YR 2023 UL http://openres.ersjournals.com/content/early/2023/03/23/23120541.00749-2022.abstract AB Background Idiopathic 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.Methods This 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).Results Of 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.Conclusion Assessment of nutritional status by GNRI provides useful information for managing patients with iPPFE by predicting mortality risk.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflicts of interest: The authors declare that no competing interests exist.