Cristina Scaletti, Sara Pratesi, Silvia Bellando Randone, Linda Di Pietro, Corrado Campochiaro, Francesco Annunziato, Marco Matucci Cerinic
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引用次数: 0
摘要
系统性硬化症是一种罕见的自身免疫性疾病,先天性免疫反应和适应性免疫反应都会发生改变,从而产生自身抗体。免疫系统的异常会损害血管的正常功能,导致血管病变,表现为雷诺现象,这是系统性硬化症的早期症状。由于这种反应性症状,疾病会发展成组织纤维化。目前已知的几种系统性硬化症特异性自身抗体与特定的临床表现和预后有关。虽然这些自身抗体的致病作用尚不明确,但它们由 B 细胞和浆细胞产生,表明这些细胞在系统性硬化症的发病过程中起着重要作用。这篇综述叙述了 B 细胞功能障碍及其在系统性硬化症发病机制中的作用,并讨论了目前用于或可能用于治疗终末器官并发症的 B 细胞靶向疗法。
The B-cells paradigm in Systemic Sclerosis: an update on pathophysiology and B-cell targeted therapies.
Systemic sclerosis is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting in the production of autoantibodies. Abnormalities of the immune system compromise the normal function of blood vessels leading to a vasculopathy manifested by Raynaud's phenomenon, an early sign of systemic sclerosis. As a consequence of this reactive picture, the disease can evolve leading to tissue fibrosis. Several systemic sclerosis-specific autoantibodies are currently known and are associated with specific clinical manifestations and prognosis. Although the pathogenetic role of these autoantibodies is still unclear, their production by B cells and plasma cells suggests the importance of these cells in the development of systemic sclerosis. This review narratively examines B cell dysfunctions and their role in the pathogenesis of systemic sclerosis and discusses B cell-targeted therapies currently used or potentially useful for the management of end-organ complications.
期刊介绍:
Clinical & Experimental Immunology (established in 1966) is an authoritative international journal publishing high-quality research studies in translational and clinical immunology that have the potential to transform our understanding of the immunopathology of human disease and/or change clinical practice.
The journal is focused on translational and clinical immunology and is among the foremost journals in this field, attracting high-quality papers from across the world. Translation is viewed as a process of applying ideas, insights and discoveries generated through scientific studies to the treatment, prevention or diagnosis of human disease. Clinical immunology has evolved as a field to encompass the application of state-of-the-art technologies such as next-generation sequencing, metagenomics and high-dimensional phenotyping to understand mechanisms that govern the outcomes of clinical trials.