ICD-11 classes
15 Diseases of the musculoskeletal system or connective...
4A60 — Monogenic autoinflammatory syndromes

ICD-11 4A60 — Monogenic autoinflammatory syndromes

Monogenic hereditary autoinflammatory diseases characterised by apparently unprovoked generalised inflammation in the absence of infection or high titre autoantibodies.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 4A60 contains 5 clarifying diagnoses:

  1. 4A60.0 — Familial Mediterranean fever
  2. 4A60.1 — Cryopyrin-associated periodic syndromes
  3. 4A60.2 — Tumour necrosis factor receptor 1 associated periodic syndrome
  4. 4A60.Y — Other specified monogenic autoinflammatory syndromes
  5. 4A60.Z — Autoimflammatory syndrome, unspecified

The diagnosis is coded elsewhere:

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