Systemic juvenile idiopathic arthritis (JIA) is a form of arthritis in childhood that is initially dominated by innate immunity-driven systemic inflammation and is thus considered a polygenic autoinflammatory disease. However, systemic JIA can progress toward an adaptive immunity-driven afebrile arthritis. Based on this observation of biphasic disease progression, a "window of opportunity" for optimal, individualized and target-directed treatment has been proposed. This hypothesis requires testing, and in this review we summarize current evidence regarding molecular factors that may contribute to the progression from an initially predominantly autoinflammatory disease phenotype to autoimmune arthritis. We consider the involvement of innately adaptive γδ T cells and natural killer T cells that express γδ or αβ T cell receptors but cannot be classified as either purely innate or adaptive cells, versus classic B and T lymphocytes in this continuum. Finally, we discuss our understanding of how and why some primarily autoinflammatory conditions can progress toward autoimmune-mediated disorders over the disease course while others do not and how this knowledge may be used to offer individualized treatment.

中文翻译：

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天生的适应性或真正的自身免疫性：系统性幼年特发性关节炎有什么独特之处吗？

系统性幼年特发性关节炎（JIA）是儿童期的一种关节炎，最初以先天性免疫驱动的全身性炎症为主，因此被认为是一种多基因性自身炎症性疾病。但是，全身性JIA可以发展为适应性免疫驱动的发热性关节炎。基于对双相性疾病进展的观察，已经提出了最佳，个性化和针对性治疗的“机会之窗”。该假设需要检验，在本综述中，我们总结了有关分子因素的最新证据，这些分子因素可能会导致从最初主要为自身炎症性疾病的表型发展为自身免疫性关节炎。我们考虑了固有适应性γδT细胞和表达γδ或αβT细胞受体但不能归类为纯天然或适应性细胞的自然杀伤性T细胞，与经典B和T淋巴细胞在此连续体中的参与。最后，我们讨论我们对某些主要的自身炎症性疾病在整个疾病过程中如何以及为什么可以发展为自身免疫介导的疾病的理解，而另一些则不能，以及如何将这些知识用于提供个性化治疗的理解。