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ORPHA:1451Disease
Also called Chronic infantile neurological cutaneous and articular syndrome · IOMID syndrome · Infantile-onset multisystem inflammatory disease · NOMID syndrome · Neonatal-onset multisystem inflammatory disease · Prieur-Griscelli syndrome
What it is
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).
Key facts
- Age of onset
- Infancy, Neonatal
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 1 000 000 (France)NLRP3-associated autoinflammatory disease
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Signs and symptoms
Very common80–99%
20- Abnormality of granulocytes
- Abnormality of neutrophils
- Arthralgia
- Brachydactyly
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- Fatigue
- Fever
- Hearing impairment
- Increased intracranial pressure
- Inflammatory abnormality of the eye
- Meningitis
- Migraine
- Myalgia
- Nausea and vomiting
- Papule
- Pseudopapilledema
- Sensorineural hearing impairment
- Urticaria
- Uveitis
Common30–79%
15Sometimes5–29%
9- Blindness
- EEG abnormality
- Global developmental delay
- Growth delay
- Intellectual disability
- Premature birth
- Purpura
- Reduced bone mineral density
and 1 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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