Abstract: Systemic autoinflammatory diseases （SAIDs） is a group of diseases caused by systemic inflammatory response due to dysfunction of the innate immune system. Due to genetic mutations causing changes in the encoded protein， it is also known as hereditary autoinflammatory disease. SAIDs have a wide variety of types， clinical manifestations and pathogenesis. It is classified into different types based on its clinical characteristics and pathogenesis. These diseases are more common in childhood and can affect multiple systems throughout the body. Among them， neurological damage is an important factor affecting the clinical prognosis of patients. Due to insufficient understanding of SAIDs， neurological damage often presents as non-specific symptoms and is easily misdiagnosed. Early identification， diagnosis， and initiation of treatment are of great significance for improving clinical prognosis. 

Key words: systemic autoinflammatory diseases, neurolo-gical damage, innate immune

摘要： 系统性自身炎症性疾病（systemic autoinflam-matory diseases，SAIDs）是固有免疫（先天免疫系统）失调引起的全身性炎症反应的一组疾病，是由基因突变引起的编码蛋白发生改变造成的，故又被称为遗传性自身炎症性疾病。SAIDs种类繁多，临床表现和发病机制多样，根据其临床特点及发病机制分为不同种类。这类疾病多于儿童期起病，可累及全身多系统，其中神经系统损害是影响患儿临床预后的重要因素。由于对SAIDs认识不足，且神经系统损害常以非特异性症状出现，因而易被误诊。应早期识别、诊断并尽早启动治疗。

关键词: 系统性自身炎症性疾病, 神经系统损伤, 固有免疫