• Respiratory medicine · Sep 2020

    Comparative Study

    Disease course and prognosis of pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis.

    • Yuzo Suzuki, Tomoyuki Fujisawa, Hiromitsu Sumikawa, Tomonori Tanaka, Chikatoshi Sugimoto, Masato Kono, Hironao Hozumi, Masato Karayama, Kazuki Furuhashi, Noriyuki Enomoto, Yutaro Nakamura, Naoki Inui, and Takafumi Suda.
    • Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. Electronic address: yuzosuzu@hama-med.ac.jp.
    • Respir Med. 2020 Sep 1; 171: 106078.

    BackgroundIdiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterized by unique radiological and pathological findings. However, pathological evaluations are available only in a limited number of patients. Therefore, several clinical diagnostic criteria have been proposed. Nevertheless, the applicability of these criteria has not yet been validated. Moreover, the clinical course of iPPFE and its prognosis have not yet been completely elucidated.MethodsThe present study assessed previously proposed clinical diagnostic criteria by comparing the clinical features between pathologically diagnosed iPPFE (p-iPPFE) and clinically diagnosed iPPFE (c-iPPFE). Subsequently, the clinical features of iPPFE were characterized and compared with those of idiopathic pulmonary fibrosis (IPF, n = 323).ResultsClinical characteristics of c-iPPFE (n = 27) and p-iPPFE (n = 35) were similar. No significant difference was observed in terms of prognosis between c-iPPFE and p-iPPFE. The number of patients with iPPFE (both c-iPPFE and p-iPPFE) who developed lung cancer was significantly lower than that of patients with IPF. However, acute exacerbation (AE) showed similar incidence in patients with iPPFE and IPF. Survival of patients with iPPFE was significantly worse than that of patients with IPF (5-year survival rate: 38.5% vs. 63.5%, p < 0.0001), and the most common cause of death was chronic respiratory failure (73.8%), followed by AE (14.3%). Male gender was the only poor prognostic factor of iPPFE.ConclusionThe present study demonstrated efficiency of clinical diagnosis and also revealed clinically important characteristics of iPPFE that should be considered for management of iPPFE.Copyright © 2020 Elsevier Ltd. All rights reserved.

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