Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama, Hamamatsu 431-3192, Japan.
Respir Med. 2013 Oct;107(10):1608-16. doi: 10.1016/j.rmed.2013.08.008. Epub 2013 Aug 29.
Elastic fibers enhance the stiffness of fibrotic tissues, but their role in the pathophysiology of idiopathic pulmonary fibrosis (IPF) has not been fully examined. The aim of this study was to determine clinical significance of the amount of elastic fibers in IPF.
We studied the surgical lung biopsy specimens of 43 patients with IPF. Histological specimens were stained using the Elastica Van Gieson method and digital images were taken. The number of pixels containing elastic fibers was divided by the number occupied by fibrotic tissue, from which the proportion of elastic fibers (elastic fiber score, %) was calculated. The relationships between the elastic fiber score and clinical, radiological and pathological findings, and prognosis were explored.
The median elastic fiber score was 10.9% (range 5.1-23.3%). Scores were inversely correlated with % predicted forced vital capacity (r -0.451, p-value 0.003) and positively correlated with decline in forced vital capacity over 12 months (r -0.475, p-value 0.033). Furthermore, elastic fiber score correlated with the extent of fibrotic lesions assessed on high resolution computed tomography as well as the degree of collagen deposition on biopsy specimens. Patients with high elastic fiber scores had significantly worse outcomes than those with low scores (5-year survival rate was 48.7% and 84.0%, respectively, p-value 0.024), and elastic fiber score was an independent predictor of poor prognosis (hazard ratio 1.21, p-value 0.005).
The amount of elastic fiber in fibrotic tissue is a prognostic indicator in patients with IPF.
弹性纤维可增强纤维组织的硬度，但它们在特发性肺纤维化（IPF）的病理生理学中的作用尚未被充分研究。本研究旨在确定弹性纤维在 IPF 中的含量的临床意义。
我们研究了 43 例 IPF 患者的手术肺活检标本。使用弹性Van Gieson 染色法对组织学标本进行染色，并拍摄数字图像。通过将含有弹性纤维的像素数除以纤维化组织所占的像素数，计算出弹性纤维的比例（弹性纤维评分，%）。探讨了弹性纤维评分与临床、影像学和病理学发现以及预后之间的关系。
中位数弹性纤维评分为 10.9%（范围 5.1-23.3%）。评分与预计用力肺活量的%呈负相关（r=-0.451，p 值=0.003），与 12 个月内用力肺活量下降呈正相关（r=-0.475，p 值=0.033）。此外，弹性纤维评分与高分辨率计算机断层扫描评估的纤维化病变程度以及活检标本中胶原沉积的程度相关。弹性纤维评分较高的患者预后明显差于评分较低的患者（5 年生存率分别为 48.7%和 84.0%，p 值=0.024），弹性纤维评分是预后不良的独立预测因子（危险比 1.21，p 值=0.005）。
纤维化组织中弹性纤维的含量是 IPF 患者的预后指标。
