neonatal onset multisystem inflammatory disease
Tuesday 13 October 2009
Neonatal Onset Multisystem Inflammatory Disease (NOMID) bears an alternate complex descriptive name, CINCA (Chronic Infantile Neurological Cutaneous and Articular syndrome). It is the most severe of the cryopyrinopathies.
Along with fevers, rash, and hearing loss, NOMID patients experience major deforming joint disease, uveitis, chronic meningitis, cerebral atrophy and delayed development. They, too, are susceptible to amyloidosis. Death within the first two decades occurs in 20% of NOMID patients.
In addition to anakinra, other therapies that may suppress disease progression include steroids, etanercept, possibly thalidomide, as well as a newly developed humanized anti IL-6 receptor monoclonal antibody.
Prieur AM. A recently recognised chronic inflammatory disease of early onset characterized by the triad of rash, central nervous system involvement and arthropathy. Clin Exp Rheumatol. 2001 Jan–Feb;19(1):103–6.