Systemic sclerosis (SSc) is an autoimmune disease which is characterized by vasculopathy, tissue fibrosis and activation of the innate and adaptive immune system. Clinical features of the disease consists of skin thickening and internal organ involvement. Due to the heterogeneous nature of the disease it is difficult to predict disease progression and complications. Despite the discovery of novel autoantibodies associated with SSc, there is an unmet need for biomarkers for diagnosis, disease progression and response to treatment. To date, the use of single (surrogate) biomarkers for these purposes has been unsuccessful. Combining multiple biomarkers in to predictive panels or ultimately algorithms could be more precise. Given the limited therapeutic options and poor prognosis of many SSc patients, a better understanding of the immune-pathofysiological profiles might aid to an adjusted therapeutic approach. Therefore, we set out to explore immunological fingerprints in various clinically defined forms of SSc. We used multilayer profiling to identify unique immune profiles underlying distinct autoantibody signatures. These immune profiles could fill the unmet need for prognosis and response to therapy in SSc. Here, we present 3 pathophysiological fingerprints in SSc based on the expression of circulating antibodies, vascular markers and immunomodulatory mediators.

中文翻译：

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全身性硬化症精准医学的诊断资料；从单一生物标志物向病理生理学小组迈进。

系统性硬化症（SSc）是一种自身免疫性疾病，其特征在于血管病变，组织纤维化以及先天性和适应性免疫系统的激活。该疾病的临床特征包括皮肤增厚和内脏受累。由于疾病的异质性，很难预测疾病的进展和并发症。尽管发现了与SSc相关的新型自身抗体，但对生物标志物的诊断，疾病进展和对治疗的反应仍未得到满足。迄今为止，用于这些目的的单一（替代）生物标志物的使用一直没有成功。将多个生物标志物结合到预测面板或最终算法中可能会更加精确。鉴于许多SSc患者的治疗选择有限且预后不良，更好地了解免疫病理组织学特征可能有助于调整治疗方法。因此，我们着手探索各种临床定义形式的SSc的免疫学指纹。我们使用了多层分析来鉴定独特的免疫特征，这些独特的免疫基础是独特的自身抗体特征。这些免疫特性可以满足SSc对预后和对治疗反应的未满足需求。在这里，我们根据循环抗体，血管标志物和免疫调节介质的表达，在SSc中呈现3种病理生理指纹。这些免疫特性可以满足SSc对预后和对治疗反应的未满足需求。在这里，我们根据循环抗体，血管标志物和免疫调节介质的表达，在SSc中呈现3种病理生理指纹。这些免疫特性可以满足SSc对预后和对治疗反应的未满足需求。在这里，我们根据循环抗体，血管标志物和免疫调节介质的表达，在SSc中呈现3种病理生理指纹。