Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias
Abstract Background Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significan...
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oai:doaj.org-article:f00df924f2a340db8a5d9bceea69728c2021-11-14T12:31:56ZRadiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias10.1186/s12931-021-01892-91465-993Xhttps://doaj.org/article/f00df924f2a340db8a5d9bceea69728c2021-11-01T00:00:00Zhttps://doi.org/10.1186/s12931-021-01892-9https://doaj.org/toc/1465-993XAbstract Background Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs. Methods A post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data. Results Of the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs. Conclusion Radiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice.Tomoyuki FujisawaYasuoki HoriikeRyoko EgashiraHiromitsu SumikawaTae IwasawaShoichiro MatsushitaHiroaki SugiuraKensuke KataokaMikiko HashisakoHideki YasuiHironao HozumiMasato KarayamaYuzo SuzukiKazuki FuruhashiNoriyuki EnomotoYutaro NakamuraNaoki InuiTakafumi SudaBMCarticlePleuroparenchymal fibroelastosisIdiopathic interstitial pneumoniasPrognosisIPFUnclassifiable IIPsDiseases of the respiratory systemRC705-779ENRespiratory Research, Vol 22, Iss 1, Pp 1-9 (2021) |
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Pleuroparenchymal fibroelastosis Idiopathic interstitial pneumonias Prognosis IPF Unclassifiable IIPs Diseases of the respiratory system RC705-779 |
| spellingShingle |
Pleuroparenchymal fibroelastosis Idiopathic interstitial pneumonias Prognosis IPF Unclassifiable IIPs Diseases of the respiratory system RC705-779 Tomoyuki Fujisawa Yasuoki Horiike Ryoko Egashira Hiromitsu Sumikawa Tae Iwasawa Shoichiro Matsushita Hiroaki Sugiura Kensuke Kataoka Mikiko Hashisako Hideki Yasui Hironao Hozumi Masato Karayama Yuzo Suzuki Kazuki Furuhashi Noriyuki Enomoto Yutaro Nakamura Naoki Inui Takafumi Suda Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| description |
Abstract Background Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs. Methods A post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data. Results Of the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs. Conclusion Radiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice. |
| format |
article |
| author |
Tomoyuki Fujisawa Yasuoki Horiike Ryoko Egashira Hiromitsu Sumikawa Tae Iwasawa Shoichiro Matsushita Hiroaki Sugiura Kensuke Kataoka Mikiko Hashisako Hideki Yasui Hironao Hozumi Masato Karayama Yuzo Suzuki Kazuki Furuhashi Noriyuki Enomoto Yutaro Nakamura Naoki Inui Takafumi Suda |
| author_facet |
Tomoyuki Fujisawa Yasuoki Horiike Ryoko Egashira Hiromitsu Sumikawa Tae Iwasawa Shoichiro Matsushita Hiroaki Sugiura Kensuke Kataoka Mikiko Hashisako Hideki Yasui Hironao Hozumi Masato Karayama Yuzo Suzuki Kazuki Furuhashi Noriyuki Enomoto Yutaro Nakamura Naoki Inui Takafumi Suda |
| author_sort |
Tomoyuki Fujisawa |
| title |
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| title_short |
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| title_full |
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| title_fullStr |
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| title_full_unstemmed |
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| title_sort |
radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias |
| publisher |
BMC |
| publishDate |
2021 |
| url |
https://doaj.org/article/f00df924f2a340db8a5d9bceea69728c |
| work_keys_str_mv |
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