Ichimura Yohei, Asano Yoshihide, Akamata Kaname, Aozasa Naohiko, Noda Shinji, Taniguchi Takashi, Takahashi Takehiro, Toyama Tetsuo, Sumida Hayakazu, Kuwano Yosihiro, Yanaba Koichi, Tada Yayoi, Sugaya Makoto, Sato Shinichi, Kadono Takafumi
Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo 113-8655, Japan.
Acta Derm Venereol. 2014 Mar;94(2):157-62. doi: 10.2340/00015555-1680.
Angiopoietin-like protein 3 (ANGPTL3), which is part of a family of secreted glycoproteins that are structurally similar to angiopoietins, is principally expressed in the liver and is involved in lipid metabolism and angiogenesis. The aim of this study was to determine the clinical significance of serum ANGPTL3 levels, measured with a specific enzyme-linked immunosorbent assay, in patients with systemic sclerosis. Serum ANGPTL3 levels correlated positively with skin score in diffuse cutaneous systemic sclerosis with a disease duration ≤ 6 years. Furthermore, the prevalence of digital ulcers was significantly higher in patients with elevated serum ANGPTL3 levels than in other patients. Moreover, among patients excluding diffuse cutaneous systemic sclerosis with disease duration ≤ 6 years, serum ANGPTL3 levels correlated positively with estimated right ventricular systolic pressure. In conclusion, ANGPTL3 may contribute to the development of progressive skin sclerosis and proliferative obliterative vasculopathy, such as digital ulcers and pulmonary vascular involvement leading to pulmonary arterial hypertension, in systemic sclerosis.
血管生成素样蛋白3（ANGPTL3）是分泌性糖蛋白家族的一员，其结构与血管生成素相似，主要在肝脏中表达，参与脂质代谢和血管生成。本研究的目的是通过特定的酶联免疫吸附测定法，确定系统性硬化症患者血清ANGPTL3水平的临床意义。血清ANGPTL3水平与疾病持续时间≤6年的弥漫性皮肤系统性硬化症患者的皮肤评分呈正相关。此外，血清ANGPTL3水平升高的患者中，指端溃疡的患病率显著高于其他患者。此外，在排除疾病持续时间≤6年的弥漫性皮肤系统性硬化症患者中，血清ANGPTL3水平与估计的右心室收缩压呈正相关。总之，在系统性硬化症中，ANGPTL3可能促进进行性皮肤硬化和增殖性闭塞性血管病的发展，如指端溃疡和导致肺动脉高压的肺血管受累。
