自身免疫,自身炎症和肾脏

S. Moiseev, N. Bulanov
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摘要

全身性自身免疫性和自身炎症性疾病与所有类型的弥漫性肾脏疾病相关,如肾小球肾炎(包括快速进展性)、血栓性微血管病、小管间质性肾炎和aa -淀粉样变性。然而,在各种全身免疫介导性炎症性疾病的患者中,肾病的发生和类型有显著差异。例如,肾小球肾炎是全身性红斑狼疮和小血管炎的主要表现之一,血栓性血管病变常见于原发性和继发性抗磷脂综合征患者,小管间质性肾炎常见于干燥综合征和igg4相关疾病患者,而aa -淀粉样变可并发类风湿关节炎、强直性脊柱炎、银屑病关节炎、家族性地中海热,以及其他一些单基因自身炎症性疾病。在过去的几十年里,由于早期诊断和有效的免疫抑制和抗炎治疗的发展,自身免疫性和自身炎症性疾病患者的肾脏存活率显著提高。然而,仍有一部分患者表现为进行性肾功能损害,这可能与基础疾病的活动无关。
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Autoimmunity, autoinflammation and kidney
Systemic autoimmune and autoinflammatory disease are associated with all types of diffuse kidney disease, that is, glomerulonephritis (including rapidly progressive), thrombotic microangiopathy, tubulointerstitial nephritis, and AA-amyloidosis. However, the occurrence and types of nephropathies differ significantly in patients with various systemic immunemediated inflammatory diseases. For example, glomerulonephritis is one of the leading manifestation of systemic lupus erythematosus and small-vessels vasculitis, thrombotic angiopathy occurs frequently in patients with both primary and secondary antiphosholipid syndrome, tubulointerstitial nephritis is frequently found in patients with Sjogren syndrome and IgG4-associated disease, whereas AA-amyloidosis can complicate rheumatoid arthritis, ankylosing spondilytis, psoriatic arthritis, familial Meditarranean fever, and certain other monogenic autoinflammatory diseases. Over last decades, renal survival in patients with autoimmune and autoinflammatory diseases improved significantly due to earlier diagnosis and development of effective immunosuppressive and anti-inflammatory treatments. However, a proportion of patients still present with progressive impairment of kidney function that can be unrelated to activity of underlying diseases.
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