关键词: 系统性自身炎症性疾病, 肾脏损伤, 肾淀粉样变性病, AA淀粉样变性

Abstract: The kidney is a major target organ of systemic autoinflammatory diseases. The clinical manifestations vary from proteinuria， nephrotic syndrome， and renal insufficiency which may end up requiring renal failure or death. The pathogenetic mechanisms are likewise quite diverse， ranging from amyloidosis caused by persistent or recurrent inflammation to inflammasome activation-mediated vasculitis/vasculopathy and several other uncommon types of glomerulonephritis. It is critical to increase the understanding and early identification of autoinflammatory diseases， as well as acquire pathology in time when renal injury occurs， to enhance disease recovery and prognosis.

Key words: systemic autoinflammatory diseases, kidney injury, renal amyloidosis, amyloid A amyloidosis