A recently recognised chronic inflammatory disease of early onset characterised by the triad of rash, central nervous system involvement and arthropathy
Am. Prieur, A recently recognised chronic inflammatory disease of early onset characterised by the triad of rash, central nervous system involvement and arthropathy, CLIN EXP RH, 19(1), 2001, pp. 103-106
Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome, al
so called Neonatal Onset Multisystemic Inflammatory Disease (NOMID) is char
acterised by the triad of cutaneous rash, chronic meningitis and arthropath
y. It is a chronic inflammatory illness that star ts most often at birth an
d persists for the whole lifespan of the patient. Attempts at therapy have
been disappointing. The long-term prognosis is poor, with progressive deafn
ess and visual impairment, and worsening of the central nervous system mani
festations. Some cases of death have been reported secondary to infection,
vasculitis and amyloidosis. Usually observed as sporadic cases, some famili
al association is recognised.