Endothelial and fibroblastic activation in scleroderma. The myth of the "uninvolved skin".
Endothelial and fibroblastic activation in scleroderma. The myth of the "uninvolved skin".
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DOI：
10.1002/art.1780341204
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发表时间：
2010-08
影响因子：
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通讯作者：
H. Claman;H. Claman;R. Giorno;R. Giorno;J. Seibold
中科院分区：
文献类型：
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作者：
H. Claman;H. Claman;R. Giorno;R. Giorno;J. Seibold
我们研究了硬皮病患者皮肤的免疫组织化学，以确定临床上“受累”和“非受累”区域之间的差异（如果有的话）。我们检查了19例弥漫性硬皮病患者和15例正常对照组的临床受累前臂皮肤（“受累”）和临床未受累近端皮肤（“未受累”）的配对皮肤活检样本。我们用内皮白细胞粘附分子1型（ELAM-1;检测内皮活化）和前胶原-1（PC-1;检测新形成的未加工胶原）的抗体对切片进行染色。与对照组相比，ELAM-1和PC-1在硬皮病皮肤中的表达增加，但在临床受累和未受累皮肤样本之间没有差异。在11名病情恶化的患者中，有10名患者的内皮细胞和成纤维细胞活化先于纤维化。内皮细胞和成纤维细胞活化在硬皮病患者的皮肤中比通过体格检查发现的更广泛。在弥漫性硬皮病中看似“正常”的皮肤已经是病理性的，如异常的内皮活化和前胶原生成所示。
We studied the immunohistochemistry of the skin of scleroderma patients to determine the differences (if any) between clinically "affected" and "nonaffected" areas. We examined paired skin biopsy samples from clinically involved forearm skin ("affected") and clinically uninvolved proximal skin ("nonaffected") taken from 19 patients with diffuse scleroderma and from 15 normal control subjects. We stained the sections with antibodies to endothelial leukocyte-adherence molecule type 1 (ELAM-1; to detect endothelial activation) and to procollagen-1 (PC-1; to detect newly formed, unprocessed collagen). There was increased expression of ELAM-1 and PC-1 in sclerodermatous skin as compared with the controls, but there was no difference between clinically affected and nonaffected skin samples. In 10 of 11 patients whose condition was getting worse, endothelial and fibroblast activation preceded fibrosis. Endothelial and fibroblast activation are more widespread in the skin of scleroderma patients than is evident by inspection on physical examination. What appears to be "normal" skin in diffuse scleroderma is already pathologic, as shown by abnormal endothelial activation and procollagen production.