4A60.1

ICD-11 MMS 2026-01

Cryopyrin-associated periodic syndromes

Description

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.

Index terms

  • Cryopyrin-associated periodic syndromes
  • CAPS - [Cryopyrin-associated periodic syndromes]
  • Cryopyrinopathies
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  • Chronic infantile neurological, cutaneous and articular syndrome
  • CINCA - [Chronic infantile neurological, cutaneous and articular] syndrome
  • IOMID - [Infantile-onset multisystem inflammatory disease]
  • Infantile-onset multisystem inflammatory disease
  • NOMID - [Neonatal-onset multisystem inflammatory disease]
  • Neonatal-onset multisystem inflammatory disease
  • Prieur-Griscelli syndrome
  • Chronic infantile neurological cutaneous and articular syndrome with CNS disease
  • Familial cold autoinflammatory syndrome
  • Familial cold urticaria
  • Muckle-Wells syndrome
  • Urticaria - deafness - amyloidosis
  • Muckle Wells Syndrome with amyloidosis
  • Muckle Wells Syndrome with neurosensorial hearing loss

Inclusions

  • Cryopyrinopathies

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A60.0 – Familial Mediterranean fever
  • 4A60.2 – Tumour necrosis factor receptor 1 associated periodic syndrome
  • 4A60.Y – Other specified monogenic autoinflammatory syndromes
  • 4A60.Z – Autoimflammatory syndrome, unspecified

Foundation entity

Cryopyrin-associated periodic syndromes – http://id.who.int/icd/entity/2139918612

Part of: 4A60: Monogenic autoinflammatory syndromes › 04: Diseases of the immune system