Serum levels of connective tissue growth factor are elevated in patients with systemic sclerosis: association with extent of skin sclerosis and severity of pulmonary fibrosis.
Serum levels of connective tissue growth factor are elevated in patients with systemic sclerosis: association with extent of skin sclerosis and severity of pulmonary fibrosis.
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发表时间：
2000
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通讯作者：
S. Sato;T. Nagaoka;M. Hasegawa;T. Tamatani;T. Nakanishi;M. Takigawa;K. Takehara
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作者：
S. Sato;T. Nagaoka;M. Hasegawa;T. Tamatani;T. Nakanishi;M. Takigawa;K. Takehara
目的探讨系统性硬化症（SSc）患者血清结缔组织生长因子（CTGF）水平及其临床相关性。方法采用ELISA法检测局限性皮肤SSc （lSSc, n = 32）、弥漫性皮肤SSc （dSSc, n = 28）、系统性红斑狼疮（SLE, n = 30）、多发性肌炎/皮肌炎（PM/DM, n = 20）和健康对照（n = 30）患者血清中CTGF的含量。结果SSc患者血清CTGF水平显著高于SLE、PM/DM患者及对照组。dSSc患者的CTGF水平明显高于lSSc患者。在CTGF的临床相关性方面，CTGF升高的SSc患者发生肺纤维化、DLCO降低、肺活量下降的频率高于CTGF正常的SSc患者。此外，SSc患者DLCO和肺活量与血清CTGF水平呈负相关和直接相关。病程1 ~ 3年的dSSc患者与病程< 1年或> 3年的dSSc患者相比，CTGF水平明显升高。结论SSc患者血清CTGF水平升高，且与皮肤硬化程度、肺纤维化严重程度相关。此外，在SSc中，CTGF的产生似乎参与了纤维化的发展或维持，而不是纤维化的开始。这些数据表明，CTGF在SSc纤维化的发展中起着关键作用。
OBJECTIVE To determine the serum levels and clinical correlation of connective tissue growth factors (CTGF) in patients with systemic sclerosis (SSc). METHODS Serum samples from patients with limited cutaneous SSc (lSSc, n = 32), diffuse cutaneous SSc (dSSc, n = 28), systemic lupus erythematosus (SLE, n = 30), polymyositis/dermatomyositis (PM/DM, n = 20), and healthy control subjects (n = 30) were examined by ELISA for detection of CTGF. RESULTS Serum CTGF levels in patients with SSc were significantly higher than those in patients with SLE or PM/DM, and in controls. CTGF levels in patients with dSSc were significantly higher than those in patients with lSSc. As for clinical correlation of CTGF, SSc patients with elevated CTGF had pulmonary fibrosis, decreased DLCO, and decreased vital capacity more frequently than those with normal CTGF levels. Further, DLCO and vital capacity were inversely and directly correlated with serum CTGF levels in patients with SSc. The dSSc patients with disease duration of 1-3 years had significantly elevated levels of CTGF compared with dSSc patients with duration < 1 year or more than 3 years. CONCLUSION Serum CTGF levels were increased in patients with SSc, and correlated with the extent of skin sclerosis and the severity of pulmonary fibrosis. In addition, it appears that production of CTGF is involved in the development or maintenance of fibrosis rather than in initiation of fibrosis in SSc. These data suggest that CTGF plays a critical role in the development of fibrosis in SSc.