Możdżan Maria, Węgiel Andrzej, Biskup Laura, Brzezińska Olga, Makowska Joanna
Department of Rheumatology, Medical University of Lodz, 90-549 Lodz, Poland.
J Clin Med. 2024 May 21;13(11):3022. doi: 10.3390/jcm13113022.
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.
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明确。其关键特征包括皮肤和内脏器官的进行性纤维化、血管病变和异常的免疫激活。虽然各种抗核抗体可作为SSc分类和预后的生物标志物，但其在器官功能障碍中的直接作用仍不清楚。抗Th/To抗体约存在于5%的SSc患者中，在疾病有限亚型患者中尤为常见。虽然这些自身抗体的存在与疾病的轻度病程相关，但它们与SSc的严重临床表现之间存在密切联系，包括间质性肺疾病、肺动脉高压和胃肠道受累。此外，这些抗体与其他临床情况的关联，尤其是与恶性肿瘤的关联，需要进一步研究。此外，疾病进程似乎受抗体、特定血清细胞因子和TLR信号通路的影响。了解抗Th/To抗体的存在、其分子层面以及对治疗方案的反应之间的关系，对于开发新型个性化治疗技术至关重要，未来研究应对此进行深入分析。
