Benfaremo Devis, Svegliati Baroni Silvia, Manfredi Lucia, Moroncini Gianluca, Gabrielli Armando
Department of Clinical and Molecular Sciences, Marche Polytechnic University, Ancona, Italy.
Eur J Rheumatol. 2020 Oct;7(Suppl 3):S181-S186. doi: 10.5152/eurjrheum.2020.19131. Epub 2020 Oct 1.
Systemic sclerosis (scleroderma, SSc) is a systemic disease characterized by vascular lesions, fibrosis, and circulating autoantibodies. A complex interplay between innate and adaptive immunity, and with regard to the latter, between humoral and cellular immunity, is believed to be involved in SSc pathogenesis. Lately, close attention has been paid to the role of B cells which, once activated, release profibrotic cytokines, promote profibrotic Th2 differentiation, and produce autoantibodies. Several novel interesting autoantibodies, targeting antigens within the extracellular matrix or on the cell surface, rather than the nuclear antigens of canonical SSc-autoantibodies, have been recently described in patients with SSc. As they show stimulatory or inhibitory activity or react with structures involved in the pathogenesis of SSc lesions, they can be considered as potentially pathogenic. In this paper, we will review those which have been better characterized.
系统性硬化症（硬皮病，SSc）是一种以血管病变、纤维化和循环自身抗体为特征的全身性疾病。先天性免疫和适应性免疫之间，以及就后者而言，体液免疫和细胞免疫之间的复杂相互作用被认为参与了SSc的发病机制。最近，人们密切关注B细胞的作用，B细胞一旦被激活，就会释放促纤维化细胞因子，促进促纤维化Th2分化，并产生自身抗体。最近在SSc患者中发现了几种新型有趣的自身抗体，它们靶向细胞外基质内或细胞表面的抗原，而不是经典SSc自身抗体的核抗原。由于它们显示出刺激或抑制活性，或与SSc病变发病机制中涉及的结构发生反应，因此可被视为潜在的致病因素。在本文中，我们将回顾那些已得到更好表征的自身抗体。
