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CINCA syndrome

Disease definition

A rare, genetic, cryopyrin-associated periodic syndrome (CAPS) characterized by neonatal onset of systemic inflammation, urticarial skin rash and arthritis/arthralgia resulting in severe arthropathy and central nervous system involvement (including chronic aseptic meningitis, brain atrophy and sensorineural hearing loss).


Classification level: Disorder
  • Synonym(s):
    • Chronic infantile neurological cutaneous and articular syndrome
    • IOMID syndrome
    • Infantile-onset multisystem inflammatory disease
    • NOMID syndrome
    • Neonatal-onset multisystem inflammatory disease
    • Prieur-Griscelli syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: Infancy, Neonatal
  • ICD-10: E85.0
  • ICD-11: 4A60.1
  • OMIM: 607115
  • UMLS: C0409818
  • MeSH: -
  • GARD: 1356
  • MedDRA: 10064568

Detailed information

General public


Disease review articles

Clinical Outcome Assessment (COA)

  • Patient-Centered Outcome Measures (PCOMs)
  • English (2023) - PROQOLIDTM

Genetic Testing

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
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