Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
复制标题
DOI：
10.1371/journal.pone.0180283
复制
发表时间：
2017
期刊：
影响因子：
3.7
通讯作者：
Suda T
中科院分区：
文献类型：
--
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
放射学胸膜实质纤维弹性增生（PPFE）样病变包括肺根尖帽可以偶尔观察到在临床设置。然而，放射学上ppfe样病变在结缔组织病（CTD）相关间质性肺疾病（ILD）中的意义尚不清楚。共纳入113例与ctd相关的ILD患者，并评估放射学ppfe样病变，其定义为双侧，上肺叶和胸膜下致密实变，胸部高分辨率计算机断层扫描伴有或不伴有胸膜增厚。评估临床、放射学和病理特征。放射学上ppfe样病变在21例（19%）患者中发现，在系统性硬化症（6/14:43%）和原发性Sjögren综合征（4/14:29%）中相对常见。有ppfe样病变的患者明显比无ppfe样病变的患者年龄更大，体重指数更低，残气量与肺活量之比更高，气胸和/或纵隔气的并发症发生率更高。21例患者中有12例放射学诊断为常规间质性肺炎（UIP）或可能的UIP型。三名接受手术肺上叶活检的患者中有两名在组织病理学上显示UIP。另一名患者在尸检中被证实有上肺叶PPFE。在临床过程中，21例患者中有13例观察到放射学ppfe样病变的进展。6例患者死亡（死亡率：29%），他们的ppfe样病变通常是进行性的。在整个队列中，我们的多因素分析发现ppfe样病变是呼吸死亡的重要危险因素（风险比：4.10,95%可信区间：1.33-12.65,p = 0.01）。在与ctd相关的ILD患者中，放射学上的PPFE样病变，不仅可能表现为PPFE，还可能表现为通常由UIP引起的根尖帽和上肺叶胸膜下纤维化，这并不罕见，且与预后不良有关。临床医生应谨慎对待这种放射学发现，特别是当它是进行性的。
Radiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD). A total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, which was defined as bilateral, upper lobe, and subpleural dense consolidations with or without pleural thickening on chest high-resolution computed tomography. The clinical, radiologic, and pathologic characteristics were evaluated. Radiologic PPFE-like lesion was found in 21 patients (19%) and were relatively frequent in those with systemic sclerosis (6/14: 43%) and primary Sjögren's syndrome (4/14: 29%). Patients with PPFE-like lesion were significantly older, had lower body mass index, higher ratio of residual volume to total lung capacity, and higher complication rate of pneumothorax and/or pneumomediastinum than those without. Twelve of the 21 patients were diagnosed radiologically as usual interstitial pneumonia (UIP) or possible UIP pattern. Two of three patients who underwent surgical lung biopsy of the upper lobes showed UIP on histopathology. Another patient was confirmed to have upper lobe PPFE on autopsy. During the clinical course, progression of the radiologic PPFE-like lesions was observed in 13 of 21 patients. Six patients died (mortality rate: 29%) and their PPFE-like lesions were commonly progressive. In the total cohort, our multivariate analysis identified the presence of PPFE-like lesion as a significant risk factor for respiratory death (hazard ratio: 4.10, 95% confidence interval: 1.33–12.65, p = 0.01). In patients with CTD-related ILD, radiologic PPFE-like lesion, which may present as not only PPFE but also apical cap and upper lobe subpleural fibrosis commonly due to UIP, was not uncommon and was associated with poor prognosis. Clinicians should be cautious with this radiologic finding, particularly when it is progressive.