■系统性硬化症(SSc)是一种相对罕见的胶原病,表现为微血管病变,在自身免疫改变的背景下,过度的皮肤和内脏纤维化。SSc中的自身免疫性血管病变早期发生,并在血管生成缺陷的情况下开始于内皮细胞活化,随后血管内膜增殖。SSc中外周微循环和大循环的改变通过血管病变明显。如雷诺现象,毛细血管扩张,肢端紫癜,数字溃疡,坏疽,外周脉搏缺乏。我们的论文详细介绍了可以在SSc中鉴定的毛细血管扩张与其他类型的免疫介导的外周血管病变之间关联的研究结果。这些外周血管病变的存在可以提供有关外周血管病变程度的信息。
■共有37例确诊为SSc,2019年2月至2020年3月从布加勒斯特的一所大学诊所招募的人员参加了一项观察性研究.我们评估了毛细血管扩张的存在,作为自身免疫性微血管病变的柱头,以及它们与SSc中可能存在的其他免疫介导的外周血管病变的关联。
■在不存在的情况下确定了毛细血管扩张的存在,但尤其是在出现肢端紫癜和数字溃疡的情况下,外周脉搏缺乏的患者几乎总是有毛细血管扩张。不到四分之一的数字溃疡患者进展为坏疽,只有一个人需要截肢,毛细血管扩张不仅存在于截肢患者中,而且存在于所有坏疽患者中。
■我们认识到毛细血管扩张可能是SSc的外周血管病变特征的临床表现,它们通常与其他外周血管病变相关,可能是SSc中坏疽性溃疡风险的一个有价值的指标.
UNASSIGNED: Systemic sclerosis (SSc) is a relatively rare collagenosis manifested as microvasculopathy, excessive cutaneous and visceral fibrosis in a background of autoimmune alteration. Autoimmune vasculopathy in SSc occurs early and begins with endothelial cell activation followed by blood vessel intimal proliferation in a context of defective angiogenesis. The alteration of peripheral micro and macrocirculation in SSc is evident through vascular lesions, such as Raynaud\'s phenomenon, telangiectasias, acrocyanosis, digital ulcers, gangrene, peripheral pulse deficiency. Our paper details the results of the study on the association between telangiectasias and other types of immune-mediated peripheral vascular lesions that can be identified in SSc. The presence of these peripheral vascular lesions can provide information about the magnitude of the peripheral vasculopathy.
UNASSIGNED: A total of 37 patients diagnosed with SSc, recruited from a university clinic in Bucharest between February 2019 and March 2020, were enrolled in an observational study. We evaluated the presence of telangiectasias, as a stigma of autoimmune microvasculopathy, and their association with other immune-mediated peripheral vascular lesions that may be present in SSc.
UNASSIGNED: The presence of telangiectasias was identified in the absence, but especially in the presence of acrocyanosis and digital ulcerations, and patients with peripheral pulse deficiency almost always had telangiectasias. Less than a quarter of the patients with digital ulcers progressed unfavorably to gangrene, and only one required amputation, telangiectasias being present not only in the patient with amputation but in all patients with gangrene.
UNASSIGNED: We appreciate that telangiectasias may be the clinical expression of peripheral vasculopathy characteristic of SSc, they can often be present in association with other peripheral vascular lesions and may represent a valuable indicator for the gangrene risk of digital ulcerations in SSc.