ICD-11 classes
14 Diseases of the skin
Inflammatory dermatoses
Autoinflammatory disorders
4A60 — Monogenic autoinflammatory syndromes
4A60.0 — Familial Mediterranean fever
ICD-11 4A60.0 — Familial Mediterranean fever
FMF is an autoinflammatory disease associated with mutations in pyrin resulting in enhanced IL1 beta production. This results in clinical attacks of inflammation in the form of fever and serositis in the form of peritoneal, pleural or synovial inflammation along with increased acute phase reactants.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: