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血管内皮生长因子缺乏是硬皮病肺疾病的特征。

A vascular endothelial growth factor deficiency characterises scleroderma lung disease.

机构信息

Correspondence to Gianfranco Ferraccioli, Catholic University, Institute of Rheumatology and Affine Sciences, Rome 00168, Italy.

出版信息

Ann Rheum Dis. 2012 Sep;71(9):1461-5. doi: 10.1136/annrheumdis-2011-200657. Epub 2012 Mar 8.

DOI:10.1136/annrheumdis-2011-200657
PMID:22402140
Abstract

OBJECTIVES

Vascular endothelial growth factor (VEGF) is thought to play an important role in systemic sclerosis (SSc) pathogenesis. It was found to be upregulated in the serum and in the affected skin of scleroderma patients. However, its involvement in scleroderma lung disease is not clear. This study aimed to evaluate VEGF concentration in the bronchoalveolar lavage fluid (BALF) of scleroderma patients with interstitial lung disease, to correlate the cytokine levels in plasma and in the lung with pulmonary functional, radiological and cellular parameters, and with the progression of lung disease.

METHODS

BALF and plasma VEGF concentrations were analysed by ELISA in 55 SSc patients with lung disease and 17 controls. Cytokine real-time PCR messenger RNA expression in alveolar macrophages was assessed. Lung involvement progression was evaluated after a 1-year follow-up.

RESULTS

VEGF was found to be significantly lower in the BALF of scleroderma patients compared with controls. The lowest concentrations were observed in SSc patients with alveolitis. A decreased VEGF expression in alveolar macrophages was found in SSc patients with alveolitis. VEGF concentration in BALF correlated inversely with the ground glass score on high-resolution CT and with BALF neutrophil cell count. Moreover, SSc patients with a lower VEGF concentration showed a worsening in the interstitial score at follow-up.

CONCLUSIONS

Scleroderma interstitial lung disease is characterised by a VEGF deficiency. Lower concentrations were found in patients with progression of lung disease.

摘要

目的

血管内皮生长因子(VEGF)被认为在系统性硬皮病(SSc)发病机制中起重要作用。研究发现,其在硬皮病患者的血清和受影响的皮肤中上调。然而,其在硬皮病肺疾病中的作用尚不清楚。本研究旨在评估间质性肺疾病硬皮病患者支气管肺泡灌洗液(BALF)中的 VEGF 浓度,将血浆和肺部细胞因子水平与肺功能、影像学和细胞参数相关联,并与肺部疾病的进展相关联。

方法

通过 ELISA 分析 55 例肺疾病硬皮病患者和 17 例对照者的 BALF 和血浆 VEGF 浓度。评估肺泡巨噬细胞中细胞因子实时 PCR 信使 RNA 表达。在 1 年随访后评估肺受累进展。

结果

与对照组相比,硬皮病患者的 BALF 中 VEGF 明显降低。在硬皮病伴间质性肺病患者中观察到最低浓度。在伴有间质性肺病的硬皮病患者中,肺泡巨噬细胞中的 VEGF 表达降低。BALF 中的 VEGF 浓度与高分辨率 CT 上的磨玻璃评分和 BALF 中性粒细胞计数呈负相关。此外,BALF 中 VEGF 浓度较低的硬皮病患者在随访中出现间质评分恶化。

结论

硬皮病间质性肺病的特征是 VEGF 缺乏。在肺疾病进展的患者中发现浓度降低。

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