Neonatal onset multisystem inflammatory disease

Neonatal onset multisystem inflammatory disease
OMIM	607115
DiseasesDB	32178

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: NOMID; chronic neurologic cutaneous and articular syndrome; CINCA; CINCA syndrome; cryopyrin- associated periodic syndrome 3; CAPS3; neonatal-onset multisystemic inflammatory disease; chronic infantile neurological, cutaneous and articular syndrome

Overview

Neonatal onset multisystem inflammatory disease (also known as NOMID, Chronic Neurologic Cutaneous and Articular Syndrome, or CINCA) is a rare genetic periodic fever syndrome which causes uncontrolled inflammation in multiple parts of the body starting in the newborn period. Symptoms include skin rashes, severe arthritis, and chronic meningitis leading to neurologic damage.

NOMID can result from a mutation in the CIAS1 gene, which helps control inflammation. Mutations in this gene also cause familial cold urticaria and Muckle-Wells syndrome. NOMID has been successfully treated with the drug anakinra.

References

• Online Mendelian Inheritance in Man (OMIM) 607115
• Goldbach-Mansky, R. et al. Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1{beta} Inhibition N Engl J Med 2006 355: 581-592.