Dziankowska-Bartkowiak Bozena, Zebrowska Agnieszka, Wagrowska-Danielewicz Małgorzata, Kobos Józef, Waszczykowska Elzbieta
Zakład Immunodermatologii, Kliniki Dermatologii UM w Łodzi.
Przegl Lek. 2009;66(12):1040-5.
Scleroderma is a connective tissue disease characterized by fibrosis confined only to skin (scleroderma circumscripta, morphea) or to skin and internal organs (systemic sclerosis, SSc) as a result of vascular changes, immune dysfunction and increased production of collagen and other extracellular matrix (ECM) components. Both types of scleroderma present clinical and histological similarities in skin changes but their pathogenic relationship is still not elucited. The aim of our study was to evaluate vascular changes in both types of scleroderma on the basis of: serum levels of gelatinases--MMP-2 and MMP-9, vascular endothelial growth factor (VEGF) and its soluble receptor 2 (sVEGFR2) and expression of CD34 antigen in skin changed samples. The following patients were involved in the study: 34 patients with SSc, 31 with morphea and 15 healthy controls. Serum levels of selected parameters were estimated by ELISA method; whereas CD34 antigen immunoexpression was performed by immunohistochemistry. Serum level of MMP-9 was statistically significantly higher in SSc and morphea patients compared to the control group (848.6 ng/ml; 844.4 ng/ml vs. 535.9 ng/ml; p<0.05). Statistical analysis revealed that mean serum levels of MMP-2 was significantly (p<0.05) higher in SSc group compared to morphea and control group (240.4 ng/ml vs. 208.1 ng/ml; 211.9 ng/ml) and VEGF was higher in morphea group compared to the control group (422.2 pg/ml vs. 188.7 pg/ml). Statistically significant difference between ratio VEGF/sVEGFR2 of mean serum levels in SSc patients and the control group (0.038 pg/ml vs. 0.018 pg/ml) and positive correlation between serum levels of MMP-9 and sVEGFR2 only in control group (r=0.143) were obtained. Possitive CD34 expression was found in vascular endothelial cells cytoplasm. Mean value of immunoexpression of CD34 was statistically significantly higher in the control group (2.8 +/- 0.42) compared to SSc group (1.59 +/- 0.69) and morphea (1.42 +/- 0.50) (p<0.001). There was no statistically significant difference between immunoexpression of CD34 in skin samples of both types of scleroderma (p=0.27). The obtained results seem to confirm pathogenic similarities in endothelial cells disturbances in both types of scleroderma--SSc and morphea.
硬皮病是一种结缔组织疾病，其特征是由于血管变化、免疫功能障碍以及胶原蛋白和其他细胞外基质（ECM）成分产生增加，导致纤维化仅局限于皮肤（局限性硬皮病、硬斑病）或累及皮肤和内脏器官（系统性硬化症，SSc）。两种类型的硬皮病在皮肤变化方面呈现出临床和组织学上的相似性，但其致病关系仍未阐明。我们研究的目的是基于以下指标评估两种类型硬皮病中的血管变化：明胶酶——基质金属蛋白酶-2（MMP-2）和基质金属蛋白酶-9（MMP-9）的血清水平、血管内皮生长因子（VEGF）及其可溶性受体2（sVEGFR2），以及皮肤病变样本中CD34抗原的表达。参与本研究的患者如下：34例系统性硬化症患者、31例硬斑病患者和15名健康对照者。通过酶联免疫吸附测定（ELISA）法评估所选参数的血清水平；而CD34抗原免疫表达则通过免疫组织化学进行。与对照组相比，系统性硬化症和硬斑病患者的MMP-9血清水平在统计学上显著更高（848.6 ng/ml；844.4 ng/ml 对比 535.9 ng/ml；p<0.05）。统计分析显示，系统性硬化症组的MMP-2平均血清水平与硬斑病组和对照组相比显著更高（p<0.05）（240.4 ng/ml 对比 208.1 ng/ml；211.9 ng/ml），硬斑病组的VEGF水平高于对照组（422.2 pg/ml 对比 188.7 pg/ml）。系统性硬化症患者与对照组的平均血清水平VEGF/sVEGFR2比值存在统计学显著差异（0.038 pg/ml 对比 0.018 pg/ml），且仅在对照组中MMP-9血清水平与sVEGFR2之间存在正相关（r=0.143）。在血管内皮细胞胞质中发现CD34阳性表达。与系统性硬化症组（1.59±0.69）和硬斑病组（1.42±0.50）相比，对照组CD34免疫表达的平均值在统计学上显著更高（2.8±0.42）（p<0.001）。两种类型硬皮病皮肤样本中CD34的免疫表达之间无统计学显著差异（p=0.27）。所获得的结果似乎证实了两种类型硬皮病——系统性硬化症和硬斑病在内皮细胞紊乱方面的致病相似性。
