4A01.21 Immune dysregulation syndromes presenting primarily with autoimmunity
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
code elsewhere
- Autoimmune polyendocrinopathy type 1 (5B00)
- Syndromic multisystem autoimmune disease due to ITCH deficiency (4A43.Y)
- Aicardi-Goutières syndrome (5C55.2)
- Spondylometaphyseal dysplasia with combined immunodeficiency (LD24.4)
synonyms
- Immune dysregulation syndromes presenting primarily with autoimmunity
- Syndrome with autoimmunity
- Immunodeficiency syndromes presenting primarily 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
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