ICD-11 classes
04 Diseases of the immune system
Autoinflammatory disorders
4A60 — Monogenic autoinflammatory syndromes
4A60.1 — Cryopyrin-associated periodic syndromes
ICD-11 4A60.1 — Cryopyrin-associated periodic syndromes
CAPS is an autoinflammatory disease associated with gain of function changes in the cryopyrin protein, resulting in inflammasome activation and enhanced IL1 beta production. This results in clinical signs and symptoms of inflammation in the form of rash, fever, joint and eye symptoms with increased acute phase reactants.
It includes 1 item.
- Cryopyrinopathies
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: