Department of Clinical Immunology, Adult and Paediatric Rheumatology, National Institute of Locomotor System Disorders and Disabilities, Budapest, Hungary.
Department of Immunology, Oslo University Hospital, Rikshospitalet and University of Oslo, Oslo, Norway.
Clin Rheumatol. 2024 Oct;43(10):3055-3072. doi: 10.1007/s10067-024-07123-y. Epub 2024 Aug 29.
Systemic sclerosis (SSc) is a progressive autoimmune disorder that mainly affects the skin. There are other clinical manifestations as renal, pulmonary, cardiovascular, and gastrointestinal tract involvements. Based on the skin involvement there are two subtypes of SSc, as limited cutaneous SSc (lSSc) which involves the acral part of the body and diffuse cutaneous SSc (dSSc) resulting in significant skin thickening of the body. Despite of the extensive research the pathomechanism is not fully clarified, how Ssc develops, moreover identifying biomarkers to predict the clinical outcome and prognosis still remains challenging. Circulating biomarkers can be crucial to define the diagnosis, to predict the prognosis and monitor the clinical course. However, only some patients are responsive to the therapy in SSc, and there is a need to reach the ideal therapy for any individual to prevent or slow down the progression in early stages of the disease. In this narrative review, our purpose was to summarize the potential biomarkers in Ssc, describe their role in the diagnosis, pathomechanism, clinical course, organ manifestations, as well as the response to the therapy. Biomarkers assessment aids in the evaluation of disease progression, and disease outcome.
系统性硬化症(SSc)是一种进行性自身免疫性疾病,主要影响皮肤。还有其他临床表现,如肾脏、肺部、心血管和胃肠道受累。根据皮肤受累情况,SSc 有两种亚型,即局限性皮肤 SSc(lSSc),累及身体的肢端部位;弥漫性皮肤 SSc(dSSc),导致身体皮肤明显增厚。尽管进行了广泛的研究,但发病机制仍未完全阐明,SSc 如何发展,以及确定预测临床结果和预后的生物标志物仍然具有挑战性。循环生物标志物对于明确诊断、预测预后和监测临床病程至关重要。然而,只有部分患者对 SSc 的治疗有反应,需要针对任何个体制定理想的治疗方案,以在疾病早期预防或减缓进展。在这篇叙述性综述中,我们的目的是总结 SSc 中潜在的生物标志物,描述它们在诊断、发病机制、临床病程、器官表现以及对治疗的反应中的作用。生物标志物评估有助于评估疾病进展和疾病结局。