4A01.21

ICD-11 MMS 2026-01

Immune dysregulation syndromes presenting primarily with autoimmunity

Index terms

  • Immune dysregulation syndromes presenting primarily with autoimmunity
  • Immunodeficiency syndromes presenting primarily with autoimmunity
  • Syndrome with autoimmunity
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  • 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

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A01.20 – Immune dysregulation syndromes with hypopigmentation
  • 4A01.22 – Immune dysregulation syndromes presenting primarily with lymphoproliferation
  • 4A01.23 – Primary haemophagocytic lymphohistiocytosis
  • 4A01.2Y – Other specified diseases of immune dysregulation
  • 4A01.2Z – Diseases of immune dysregulation, unspecified

Foundation entity

Immune dysregulation syndromes presenting primarily with autoimmunity – http://id.who.int/icd/entity/1902856995

Part of: 4A01.2: Diseases of immune dysregulation › 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system