狼疮性肾炎发病机制研究进展

Advancements in the Pathogenesis of Lupus Nephritis

系统性红斑狼疮(systemic lupus erythematosus, SLE)是一种全身性自身免疫性疾病,狼疮性肾炎(lupus nephritis, LN)是SLE最严重的脏器损害之一,也是SLE患者疾病进展或死亡的重要原因。大多数患者在诊断为SLE后会在5年内出现LN,而诊断为LN的患者中又有5%~20%会在10年内发展为终末期肾病(end stage kidney disease, ESKD)。因此,LN的早期诊断和干预对改善患者预后,提高患者生活质量至关重要。LN的发病机制给LN的早期诊治提供了重要的基础和前提。LN是典型的免疫复合物性肾炎。多种致病因素导致机体免疫耐受性被破坏,产生针对自身抗原的抗体,引起免疫复合物形成并在肾脏沉积,激活肾内免疫,造成肾脏损害。本文从遗传、环境、免疫等方面回顾并更新了LN的主要发病机制,以期为该疾病的防治提供新的策略。

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the devel-opment of lupus nephritis (LN), which represents a severe form of organ damage and a significant contributor to disease progression and mortality in SLE patients. The majority of individuals diag-nosed with SLE will develop LN within five years, while 5%~20% of those with LN will progress to end-stage kidney disease (ESKD) within ten years. Therefore, early diagnosis and intervention for LN are crucial in improving patient prognosis and quality of life. Understanding the pathogenesis of LN serves as the foundation for its timely diagnosis and treatment. As immune complex nephritis, various pathogenic factors disrupt immune tolerance, leading to autoantibody production against self-antigens, formation and deposition of immune complexes in the kidneys, as well as activation of intrarenal immunity resulting in renal damage. This review aims to provide updated insights into the hereditary, environmental, and immunological aspects underlying LN pathogenesis, offering novel strategies for its prevention and management.