Neonatal onset multisystem inflammatory disease

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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

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.