Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF).

Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF).
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DOI:
10.1186/1471-2466-14-91
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发表时间:
2014-05-28
影响因子:
3.1
通讯作者:
Suda T
Suda T
中科院分区:
医学3区
文献类型:
--
作者:
Enomoto N;Kusagaya H;Oyama Y;Kono M;Kaida Y;Kuroishi S;Hashimoto D;Fujisawa T;Yokomura K;Inui N;Nakamura Y;Suda T

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苏木精-伊红染色的特发性胸膜实质纤维弹性组织增生症(IPPFE)的病理学表现与特发性肺纤维化(IPF)患者的普通型间质性肺炎(UIP)相似。弹性纤维(EF)的数量以及IPPFE和IPF之间的详细差异尚未完全阐明。本研究的目的是量化EF并确定IPPFE和IPF之间的差异。我们评估了6例IPPFE患者和28例IPF患者,他们接受了手术肺活检或尸检。对患者的临床病史、体格检查、胸部高分辨率计算机断层扫描(HRCT)检查结果和肺标本的病理特征进行回顾性评价。使用带有电荷耦合器件的照相机和分析软件,用Weigert染色定量两组肺标本中的EF量。IPPFE患者出现细爆裂声的人数少于IPF患者(50.0%与96.4%,p = 0.012)。IPPFE患者的用力肺活量较低(62.7 ± 10.9% vs. 88.6 ± 21.9%预测值,p = 0.009),HRCT实变评分较高(1.7 ± 0.8 vs. 0.3 ± 0.5,p < 0.0001),较低的体重指数(17.9 ± 0.9 vs. 24.3 ± 2.8,p < 0.0001),气胸发生率高于IPF患者(66.7 vs. 3.6%,p = 0.002)。IPPFE患者肺标本的EF值是IPF患者的两倍多(28.5 ± 3.3% vs. 12.1 ± 4.4%,p < 0.0001)。下叶的EF量显著低于上叶,即使在同一名IPPFE患者中也是如此(23.6 ± 2.4% vs. 32.4 ± 5.5%,p = 0.048)。然而,IPPFE患者下叶的EF量仍高于IPF患者(23.6 ± 2.4% vs. 12.2 ± 4.4%,p < 0.0001)。IPPFE患者的EF量是IPF患者的两倍以上。即使在下叶,IPPFE患者的EF量也高于IPF患者,尽管IPPFE标本的肺EF分布不均匀。
The pathological appearance of idiopathic pleuroparenchymal fibroelastosis (IPPFE) with hematoxylin-eosin staining is similar to that of usual interstitial pneumonia (UIP) in patients with idiopathic pulmonary fibrosis (IPF). The amount of elastic fibers (EF) and detailed differences between IPPFE and IPF have not been fully elucidated. The aim of this study was to quantify the EF and identify the differences between IPPFE and IPF. We evaluated six patients with IPPFE and 28 patients with IPF who underwent surgical lung biopsy or autopsy. The patients’ clinical history, physical findings, chest high-resolution computed tomography (HRCT) findings, and pathological features of lung specimens were retrospectively evaluated. The amounts of EF in lung specimens were quantified with Weigert’s staining using a camera with a charge-coupled device and analytic software in both groups. Fewer patients with IPPFE than IPF had fine crackles (50.0% vs. 96.4%, p = 0.012). Patients with IPPFE had a lower forced vital capacity (62.7 ± 10.9% vs. 88.6 ± 21.9% predicted, p = 0.009), higher consolidation scores on HRCT (1.7 ± 0.8 vs. 0.3 ± 0.5, p < 0.0001), lower body mass indices (17.9 ± 0.9 vs. 24.3 ± 2.8, p < 0.0001), and more pneumothoraces than did patients with IPF (66.7 vs. 3.6%, p = 0.002). Lung specimens from patients with IPPFE had more than twice the amount of EF than did those from patients with IPF (28.5 ± 3.3% vs. 12.1 ± 4.4%, p < 0.0001). The amount of EF in the lower lobes was significantly lower than that in the upper lobes, even in the same patient with IPPFE (23.6 ± 2.4% vs. 32.4 ± 5.5%, p = 0.048). However, the amount of EF in the lower lobes of patients with IPPFE was still higher than that of patients with IPF (23.6 ± 2.4% vs. 12.2 ± 4.4%, p < 0.0001). More than twice the amount of EF was found in patients with IPPFE than in those with IPF. Even in the lower lobes, the amount of EF was higher in patients with IPPFE than in those with IPF, although the distribution of lung EF was heterogeneous in IPPFE specimens.
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发表时间: 2012-08-01
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