Area Gastroenterologia e Oncologia Medica, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Università Cattolica Sacro Cuore, Rome, Italy.
Autoimmun Rev. 2019 May;18(5):484-492. doi: 10.1016/j.autrev.2019.03.003. Epub 2019 Mar 4.
The study of free light chains (FLCs) has grown as area of enormous interest for many clinicians with the aim of disclosing the exact biological role and potential use of FLCs in the clinical routine. Moreover, the attention given to immunological functions of FLCs has sparked a new light into their pathogenic contribution in different chronic autoimmune-based inflammatory diseases. The release of intracellular antigens following cell death or ineffective clearance of apoptotic debris, modification of self-antigens, and molecular mimicry may trigger the production of immunoglobulins after activation and polyclonal expansion of B cells, by which FLCs are released. The discovery of polyclonal FLCs as potential biomarkers started with the observation of their increased concentrations in a variety of biological fluids related to patients with autoimmune diseases. This review deals with the use of polyclonal FLCs for identifying severity and monitoring outcome after treatment in some autoimmune diseases, namely systemic lupus erythematosus, myasthenia gravis, systemic sclerosis, rheumatoid arthritis and Sjögren's syndrome, as supported by the fact that levels of FLCs correlate with both B cell activation markers and other specific markers of disease activity. In a near future, following the evidence shown, FLCs might probably work as early prognostic markers of severity and also as indicators of response to treatment or early assessment of relapse in selected autoimmune diseases.
对许多临床医生来说，游离轻链（FLC）的研究已经成为一个非常感兴趣的领域，目的是揭示 FLC 在临床常规中的确切生物学作用和潜在用途。此外，对 FLC 免疫功能的关注激发了人们对其在不同慢性自身免疫性炎症性疾病中的致病作用的新认识。细胞死亡或无效清除细胞凋亡碎片后细胞内抗原的释放、自身抗原的修饰以及分子模拟可能会在 B 细胞激活和多克隆扩增后触发免疫球蛋白的产生，从而释放 FLC。多克隆 FLC 作为潜在生物标志物的发现始于观察到它们在与自身免疫性疾病患者相关的多种生物液中浓度升高。这篇综述讨论了在一些自身免疫性疾病中，使用多克隆 FLC 来识别严重程度和监测治疗后的结果，这些疾病包括系统性红斑狼疮、重症肌无力、系统性硬化症、类风湿关节炎和干燥综合征，因为 FLC 水平与 B 细胞激活标志物和其他疾病活动的特定标志物相关。在不久的将来，根据所显示的证据，FLC 可能成为严重程度的早期预后标志物，也可能成为治疗反应或在选定的自身免疫性疾病中早期评估复发的指标。
