4A01.21

自身免疫为主要表现的免疫失调综合征

Immune dysregulation syndromes presenting primarily with autoimmunity

跨章引用同一概念在其他章节另有编码

英文词条（4）

英文词条（13）

Immune dysregulation syndromes presenting primarily with autoimmunity
Immunodeficiency syndromes presenting primarily with autoimmunity
Syndrome with autoimmunity
FADD-related immunodeficiency
X-linked immune dysregulation – polyendocrinopathy – enteropathy
Autoimmune enteropathy type 1
IPEX - [immunodysregulation polyendocrinopathy enteropathy X-linked syndrome]
immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome
Immunodeficiency due to CD25 deficiency
SCID - [severe combined immunodeficiency] due to interleukin-2 receptor alpha chain deficiency
LRBA deficiency
LRBA deficiency - [Lipopolysaccharide-responsive, beige-like anchor protein] deficiency
CTLA4-Mutation

该条目未定义后组配轴，直接使用上面的编码即可。后组配是在主干编码之后追加侧别、具体解剖部位、严重程度、病因等细节的可选组合方式，不是必填项。