Department of Environmental Immuno-Dermatology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, Kanagawa 236-0004, Japan.
Br J Dermatol. 2013 Apr;168(4):717-25. doi: 10.1111/bjd.12117. Epub 2013 Mar 7.
Periostin, a matricellular protein, serves as a regulator of wound healing and fibrosis. The role of periostin in the pathogenesis of systemic sclerosis (SSc) is unknown.
To determine periostin levels in association with severity of skin fibrosis in patients with SSc.
Expression of periostin was immunohistochemically examined in skin obtained from patients with SSc and healthy controls. Enzyme-linked immunosorbent assay was performed to evaluate serum periostin levels in association with clinical characteristics in 56 patients with SSc [diffuse cutaneous SSc (dSSc), n=16; and limited cutaneous SSc (lSSc), n=40] and 66 healthy controls.
Periostin was strongly expressed in the affected dermis from patients with SSc. Periostin was colocalized in α-smooth muscle actin-positive myofibroblasts and platelet endothelial cell adhesion molecule-1-positive endothelial cells in SSc dermis. Serum levels of periostin in patients with dSSc were markedly elevated compared with those in patients with lSSc and control subjects. Patients with lSSc had increased periostin levels compared with healthy controls. In addition, significantly higher levels of periostin were observed in patients with dSSc with disease duration ≤5 years compared with those with disease duration >5 years. Furthermore, the modified Rodnan total skin thickness score (MRSS) was positively correlated with periostin levels in patients with SSc. Serial analysis revealed a correlation between periostin and MRSS; namely, MRSS decreased in line with decreased periostin levels in some patients with dSSc as the disease progressed.
An elevated periostin level in patients with SSc is associated with severity of skin sclerosis. Periostin may be a potential biomarker for progressive skin fibrosis in SSc.
细胞外基质蛋白骨桥蛋白作为一种调节因子，参与创伤愈合和纤维化过程。骨桥蛋白在系统性硬化症（SSc）发病机制中的作用尚不清楚。
确定骨桥蛋白与 SSc 患者皮肤纤维化严重程度的相关性。
采用免疫组织化学方法检测 SSc 患者和健康对照者皮肤中骨桥蛋白的表达，采用酶联免疫吸附试验检测 56 例 SSc 患者（弥漫性皮肤型 SSc，dSSc，16 例；局限性皮肤型 SSc，lSSc，40 例）和 66 例健康对照者血清骨桥蛋白水平，并与临床特征进行相关性分析。
骨桥蛋白在 SSc 患者皮损真皮层中强表达，与 SSc 皮损中α-平滑肌肌动蛋白阳性肌成纤维细胞和血小板内皮细胞黏附分子-1 阳性内皮细胞共定位。dSSc 患者血清骨桥蛋白水平明显高于 lSSc 患者和健康对照者，lSSc 患者血清骨桥蛋白水平高于健康对照者。此外，病程≤5 年的 dSSc 患者血清骨桥蛋白水平显著高于病程>5 年的患者。此外，在 SSc 患者中，改良 Rodnan 皮肤总厚度评分（MRSS）与骨桥蛋白水平呈正相关。序列分析显示骨桥蛋白与 MRSS 相关；即一些 dSSc 患者随着疾病进展，MRSS 下降，同时骨桥蛋白水平下降。
SSc 患者骨桥蛋白水平升高与皮肤硬化严重程度相关。骨桥蛋白可能是 SSc 进行性皮肤纤维化的潜在生物标志物。
