College of Medicine, Medical University of South Carolina, Charleston, SC, United States.
Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, SC, United States.
Front Immunol. 2020 Sep 25;11:586737. doi: 10.3389/fimmu.2020.586737. eCollection 2020.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that involves multiple organs and disproportionality affects females, especially African Americans from 15 to 44 years of age. SLE can lead to end organ damage including kidneys, lungs, cardiovascular and neuropsychiatric systems, with cardiovascular complications being the primary cause of death. Usually, SLE is diagnosed and its activity is assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), Systemic Lupus International Collaborating Clinics Damage Index (SLICC/ACR), and British Isles Lupus Assessment Group (BILAG) Scales, which unfortunately often occurs after a certain degree of systemic involvements, disease activity or organ damage already exists. There is certainly a need for the identification of early biomarkers to diagnose and assess disease activity as well as to evaluate disease prognosis and response to treatment earlier in the course of the disease. Here we review advancements made in the area of sphingolipidomics as a diagnostic/prognostic tool for SLE and its co-morbidities. We also discuss recent reports on differential sphingolipid metabolism and blood sphingolipid profiles in SLE-prone animal models as well as in diverse cohorts of SLE patients. In addition, we address targeting sphingolipids and their metabolism as a method of treating SLE and some of its complications. Although such treatments have already shown promise in preventing organ-specific pathology caused by SLE, further investigational studies and clinical trials are warranted.
系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,涉及多个器官,且不成比例地影响女性,尤其是 15 至 44 岁的非裔美国人。SLE 可导致终末器官损伤,包括肾脏、肺部、心血管和神经精神系统,心血管并发症是主要的死亡原因。通常,使用系统性红斑狼疮疾病活动指数(SLEDAI)、系统性红斑狼疮国际合作临床损伤指数(SLICC/ACR)和不列颠群岛狼疮评估组(BILAG)量表来诊断 SLE 及其活动,并评估其活动,不幸的是,这些通常是在已经存在一定程度的系统性受累、疾病活动或器官损伤之后才进行的。因此,确实需要识别早期生物标志物来更早地诊断和评估疾病活动以及评估疾病预后和对治疗的反应。在这里,我们回顾了 Sphingolipidomics 在 SLE 及其合并症的诊断/预后工具方面的进展。我们还讨论了最近关于 SLE 易感动物模型以及不同 SLE 患者群体中差异的鞘脂代谢和血液鞘脂谱的报告。此外,我们还讨论了靶向鞘脂及其代谢作为治疗 SLE 及其某些并发症的方法。尽管这些治疗方法已经显示出在预防由 SLE 引起的器官特异性病理学方面有一定的前景,但仍需要进一步的研究和临床试验。