A recently recognised chronic inflammatory disease of early onset characterised by the triad of rash, central nervous system involvement and arthropathy.
Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome, also called Neonatal Onset Multisystemic Inflammatory Disease (NOMID) is characterised by the triad of cutaneous rash, chronic meningitis and arthropathy. It is a chronic inflammatory illness that starts most often at birth and persists for the whole lifespan of the patient. Attempts at therapy have been disappointing. The long-term prognosis is poor, with progressive deafness and visual impairment, and worsening of the central nervous system manifestations. Some cases of death have been reported secondary to infection, vasculitis and amyloidosis. Usually observed as sporadic cases, some familial association is recognised.
Prieur
A M
AM
Pediatric Immunology, Hematology and Rheumatology Unit, Hôpital Necker-Enfants Malades, AP-HP, University Paris V, Rue de Sèvres no. 149, F-75743 Paris, France.
D017668:Age of Onset; D001171:Arthritis, Juvenile / Q000150:complications; D001177:Arthropathy, Neurogenic / Q000209:etiology*; D002493:Central Nervous System Diseases / Q000151:congenital; D002908:Chronic Disease; D005076:Exanthema / Q000209:etiology*; D006801:Humans; D007223:Infant; D007229:Infant Welfare; D007231:Infant, Newborn; D007249:Inflammation / Q000150:complications*; D012737:Sex Factors; D013577:Syndrome