Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias.
Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias.
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DOI：
10.1186/s12931-021-01892-9
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发表时间：
2021-11-11
影响因子：
5.8
通讯作者：
Suda T
中科院分区：
文献类型：
--
作者：
Fujisawa T;Horiike Y;Egashira R;Sumikawa H;Iwasawa T;Matsushita S;Sugiura H;Kataoka K;Hashisako M;Yasui H;Hozumi H;Karayama M;Suzuki Y;Furuhashi K;Enomoto N;Nakamura Y;Inui N;Suda T
胸膜实质弹力纤维增生症（PPFE）的特点是主要是上叶胸膜和胸膜下肺实质纤维化。放射学 PPFE 样病变与各种类型的间质性肺疾病有关。然而，特发性间质性肺炎（IIP）患者中放射学 PPFE 样病变的患病率和临床意义尚不完全清楚。我们的目的是确定 IIP 患者放射学 PPFE 样病变的患病率及其对生存的临床影响。使用日本全国 IIP 患者云数据库的数据进行了事后分析。数据库中的所有患者均经多学科讨论诊断为 IIP。诊断为特发性 PPFE 的患者被排除在外。分析了 419 例 IIP 患者的临床数据和胸部计算机断层扫描 (CT) 图像。放射学 PPFE 样病变的存在由两名不了解临床数据的胸部放射科医生独立评估。在 419 例 IIP 患者中，101 例（24.1%）患者检测到放射学 PPFE 样病变，主要是特发性肺纤维化（IPF）和无法分类的 IIP，但特发性非特异性间质性肺炎较少。预后分析显示，放射学 PPFE 样病变与 IIP 患者的不良预后显着相关，且与年龄、IPF 诊断和 %FVC 无关。在生存分析中，与没有放射学 PPFE 样病变的患者相比，具有放射学 PPFE 样病变的患者的生存率较差（对数秩，p<0.0001）。亚组分析表明，放射学 PPFE 样病变与 IPF 患者和无法分类的 IIP 患者的不良生存率显着相关。放射学 PPFE 样病变是 IIP 中可能存在的一种病症，主要存在于 IPF 和无法分类的 IIP 中。重要的是，放射学 PPFE 样病变是预测 IIP 患者不良预后的非侵入性标志物，在临床实践中应仔细考虑。在线版本包含可在 10.1186/s12931-021-01892-9 获取的补充材料。
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. 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. 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. 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. The online version contains supplementary material available at 10.1186/s12931-021-01892-9.
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影响因子：
4.3
作者：
Jacob, Joseph;Odink, Arlette;Wells, Athol U.
通讯作者：
Wells, Athol U.
通过手术肺活检通过多学科讨论诊断的间质性肺炎患者的胸膜纤维纤维舒张样病变。
影响因子：
2
作者：
Sumikawa H;Johkoh T;Egashira R;Sugiura H;Yamano Y;Kataoka K;Kondoh Y;Arakawa H;Nakamura M;Kuriu A;Nakanishi K;Tomiyama N
通讯作者：
Tomiyama N
影响因子：
3.7
作者：
Enomoto Y;Nakamura Y;Colby TV;Johkoh T;Sumikawa H;Nishimoto K;Yoshimura K;Matsushima S;Oyama Y;Hozumi H;Kono M;Fujisawa T;Enomoto N;Inui N;Iwashita T;Suda T
通讯作者：
Suda T
影响因子：
7.5
作者：
von der Thuesen, Jan H.;Hansell, David M.;Nicholson, Andrew G.
通讯作者：
Nicholson, Andrew G.
影响因子：
24.3
作者：
Ryerson, Christopher J.;Urbania, Thomas H.;Collard, Harold R.
通讯作者：
Collard, Harold R.