ICD-11 classes
04 Diseases of the immune system
Primary immunodeficiencies
4A00 — Primary immunodeficiencies due to disorders of innate...
4A00.1 — Defects in the complement system
4A00.14 — Hereditary angioedema
ICD-11 4A00.14 — Hereditary angioedema
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.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: