Department of Rheumatology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Department of Rheumatology, People's Hospital of Leshan, Sichuan, China.
Clin Exp Rheumatol. 2021 Jan-Feb;39(1):151-157. doi: 10.55563/clinexprheumatol/ubov8b. Epub 2020 Sep 3.
We aimed to reveal evidence of endothelial dysfunction in the development of anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis (DM).
Thirty anti-MDA5 DM patients were enrolled and compared with patients with polymyositis (PM) (n=10) and healthy controls (n=20). The concentrations of soluble intercellular adhesion molecule-1 (sICAM-1), soluble vascular cell adhesion molecule-1 (sVCAM-1), endothelin-1 (ET-1) and von Willebrand factor (vWF) as well as interferon-alpha (IFN-α) and Galcetin-9 in the peripheral blood were tested by enzyme-linked immunosorbent assay (ELISA).
Plasma levels of sICAM-1, sVCAM-1, ET-1 and vWF were higher in the anti-MDA5 DM patients than in either the healthy controls or the PM patients. In the anti-MDA5 DM cohort, the ET-1 and vWF levels were significantly lower in the cases without cutaneous ulcers and ILD than the other cases. There was a strong positive relationship between the concentrations of ET-1 and Galectin-9 in the anti-MDA5 DM group.
Our data suggest that endothelial dysfunction may be involved in the development of anti-MDA5 DM.
我们旨在揭示抗黑色素瘤分化相关基因 5(MDA5)皮肌炎(DM)发病过程中内皮功能障碍的证据。
纳入 30 名抗 MDA5 DM 患者,并与多发性肌炎(PM)患者(n=10)和健康对照者(n=20)进行比较。采用酶联免疫吸附试验(ELISA)检测外周血中可溶性细胞间黏附分子-1(sICAM-1)、可溶性血管细胞黏附分子-1(sVCAM-1)、内皮素-1(ET-1)、血管性血友病因子(vWF)、干扰素-α(IFN-α)和 Galcetin-9 的浓度。
与健康对照者或 PM 患者相比,抗 MDA5 DM 患者的血浆 sICAM-1、sVCAM-1、ET-1 和 vWF 水平更高。在抗 MDA5 DM 队列中,无皮肤溃疡和ILD 的患者的 ET-1 和 vWF 水平显著低于其他患者。抗 MDA5 DM 组中 ET-1 和 Galectin-9 浓度之间存在强正相关关系。
我们的数据表明,内皮功能障碍可能参与了抗 MDA5 DM 的发病。