A recently recognised chronic inflammatory disease of early onset characterised by the triad of rash, central nervous system involvement and arthropathy

Authors
Citation
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
Citations number
33
Categorie Soggetti
Rheumatology,"da verificare
Journal title
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
ISSN journal
0392856X → ACNP
Volume
19
Issue
1
Year of publication
2001
Pages
103 - 106
Database
ISI
SICI code
0392-856X(200101/02)19:1<103:ARRCID>2.0.ZU;2-#
Abstract
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.