PDF(Pubmed)
Abstract：
Systemic sclerosis (SSc) represents a rare and intricate autoimmune connective tissue disease, the pathophysiology of which has not been fully understood. Its key features include progressive fibrosis of the skin and internal organs, vasculopathy and aberrant immune activation. While various anti-nuclear antibodies can serve as biomarkers for the classification and prognosis of SSc, their direct role in organ dysfunction remains unclear. Anti-Th/To antibodies are present in approximately 5% of SSc patients, and are particularly prevalent among those with the limited subtype of the disease. Although the presence of these autoantibodies is associated with a mild course of the disease, there is a strong connection between them and severe clinical manifestations of SSc, including interstitial lung disease, pulmonary arterial hypertension and gastrointestinal involvement. Also, the additional clinical correlations, particularly with malignancies, need further research. Moreover, the disease\'s course seems to be influenced by antibodies, specific serum cytokines and TLR signaling pathways. Understanding the relationships between presence of anti-Th/To, its molecular aspects and response to treatment options is crucial for the development of novel, personalized therapeutic techniques and should undergo profound analysis in future studies.
摘要：
系统性硬化症（SSc）是一种罕见且复杂的自身免疫性结缔组织疾病，其病理生理学尚未完全理解。它的主要特征包括皮肤和内脏器官的进行性纤维化，血管病变和异常免疫激活。虽然各种抗核抗体可以作为SSc分类和预后的生物标志物，它们在器官功能障碍中的直接作用尚不清楚.抗Th/To抗体存在于大约5%的SSc患者中，在这种疾病亚型有限的人群中尤其普遍。尽管这些自身抗体的存在与轻度病程有关，它们与SSc的严重临床表现之间有很强的联系，包括间质性肺病,肺动脉高压和胃肠道受累。此外，额外的临床相关性，特别是恶性肿瘤，需要进一步研究。此外，这种疾病的病程似乎受到抗体的影响，特异性血清细胞因子和TLR信号通路。理解反Th/To的存在之间的关系，它的分子方面和对治疗方案的反应对于新型药物的开发至关重要，个性化的治疗技术，并应在未来的研究中进行深刻的分析。
