4A00.14

ICD-11 MMS 2026-01

Hereditary angioedema

Description

Hereditary angioedema is caused in the majority of cases by genetically determined low absolute (type I) or functional (type II) levels of C1 inhibitor, a plasma proteinase inhibitor involved in regulation of complement activation. It is characterised clinically by recurrent subcutaneous and/or submucosal oedema and can result in life-threatening laryngeal obstruction. Involvement of the digestive tract commonly causes abdominal pain. This and the absence of accompanying urticarial weals or itch distinguish it from the common form of angioedema, which is part of the spectrum of urticaria.

Index terms

  • Hereditary angioedema
  • Bannister disease, hereditary
  • Familial angioedema
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  • HAE - [hereditary angioneurotic oedema]
  • Hereditary angioneurotic oedema
  • Quincke disease or oedema
  • hereditary Quincke oedema
  • Hereditary angioedema type I
  • C1 esterase inhibitor [C1-INH] quantitative deficiency
  • C1 esterase inhibitor deficiency - type 1
  • HAE-I - [Hereditary angioedema type I]
  • Hereditary angioneurotic oedema type 1
  • deficiency of c1 esterase inhibitor
  • Hereditary angioedema type II
  • C1 esterase inhibitor [C1-INH] qualitative deficiency
  • C1-INH - [C1 esterase inhibitor] deficiency
  • HAE-II - [Hereditary angioedema type II]
  • Hereditary angioneurotic oedema type 2
  • Hereditary angioedema type III
  • HAE-III - [Hereditary angioedema type III]
  • Hereditary angioedema with FXII mutation
  • Hereditary angioneurotic oedema type 3
  • Inherited oestrogen-dependent angioedema
  • Inherited oestrogen-dependent angioneurotic oedema
  • Acute hereditary circumscribed oedema
  • Acute hereditary essential oedema
  • Hereditary giant urticaria
  • Periodic hereditary oedema
  • Hereditary allergic angioedema
  • Hereditary angioneurotic oedema urticaria

Exclusions from above levels

  • 3A10.Y – Atypical haemolytic uraemic syndrome
  • 3A21.0 – Paroxysmal nocturnal haemoglobinuria
  • 02 – Neoplasms
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A00.10 – Immunodeficiency with an early component of complement deficiency
  • 4A00.11 – Immunodeficiency with a late component of complement deficiency
  • 4A00.12 – Immunodeficiency with factor B deficiency
  • 4A00.13 – Immunodeficiency with factor D anomaly
  • 4A00.15 – Acquired angioedema
  • 4A00.1Y – Other specified defects in the complement system
  • 4A00.1Z – Defects in the complement system, unspecified

Foundation entity

Hereditary angioedema – http://id.who.int/icd/entity/795969334

Part of: 4A00.1: Defects in the complement system › 4A00: Primary immunodeficiencies due to disorders of innate immunity › 04: Diseases of the immune system