N-terminal connective tissue growth factor is a marker of the fibrotic phenotype in scleroderma
N-terminal connective tissue growth factor is a marker of the fibrotic phenotype in scleroderma
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DOI：
10.1093/qjmed/hci078
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发表时间：
2005-07-01
影响因子：
13.3
通讯作者：
Stratton, R
中科院分区：
文献类型：
--
作者：
Dziadzio, M;Usinger, W;Stratton, R
背景：结缔组织生长因子（CTGF）的过度表达是包括硬皮病在内的纤维化疾病的标志。CTGF与促纤维化细胞因子TGF β一起作用以促进体内持续的纤维化反应。CTGF的产生增加可能是维持硬皮病纤维化表型的原因。CTGF或其片段的分析是潜在的非侵入性措施的纤维化反应在scleroderma.Aim：To determine the utility of whole，N-terminal，and C-terminal CTGF as surrogate marker for fibrosis in scleroderma.Design：Cross-sectional controlled study.Methods：血浆前瞻性收集47例硬皮病患者（26弥漫性硬皮病，21局限性硬皮病）和18例健康对照。同时，通过抽吸水泡技术从硬皮病患者的病变皮肤和健康对照者的前臂皮肤中提取真皮间质液。整个，N-末端，和C-末端CTGF的ELISA测定，使用单克隆抗体的N-和C-末端epitopes.Results：N-末端CTGF裂解产物存在于硬皮病患者的血浆和真皮间质液中的水平升高，相比健康对照。血浆和真皮间质液中的N-末端CTGF水平与皮肤疾病的严重程度和疾病持续时间呈负相关。整个和C-末端CTGF水平低水疱液和血浆水平没有升高diseases.Discussion：这些结果支持的作用，CTGF在硬皮病相关的纤维化和效用的N-末端CTGF作为纤维化的标志物。
Background: Over-expression of connective tissue growth factor (CTGF) is a hallmark of fibrotic disease, including scleroderma. CTGF acts with the pro-fibrotic cytokine TGF beta to promote sustained fibrotic responses in vivo. Elevated production of CTGF might be responsible for maintenance of the fibrotic phenotype in scleroderma. Assays of CTGF or of its fragments are potential non-invasive measures of the fibrotic response in scleroderma.Aim: To determine the utility of whole, N-terminal, and C-terminal CTGF as surrogate markers for fibrosis in scleroderma.Design: Cross-sectional controlled study.Methods: Plasma was collected prospectively from 47 scleroderma patients (26 diffuse scleroderma, 21 limited scleroderma) and 18 healthy controls. At the same time, dermal interstitial fluid was derived by a suction blister technique from the lesional skin of scleroderma patients, and from the forearm skin of healthy controls. Whole, N-terminal, and C-terminal CTGF were assayed by ELISA, using monoclonal antibodies specific for N- and C-terminal epitopes.Results: N-terminal cleavage products of CTGF were present at elevated levels in the plasma and dermal interstitial fluid of scleroderma patients, compared to healthy controls. N-terminal CTGF levels in plasma and dermal interstitial fluid correlated with severity of skin disease and (negatively) with disease duration. Whole and C-terminal CTGF levels were low in blister fluid and plasma levels were not elevated in disease.Discussion: These results support a role for CTGF in scleroderma-associated fibrosis and the utility of N-terminal CTGF as a marker of fibrosis.