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PMID: 11247311 Published · ppublish English Journal Article Review

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

Clinical and experimental rheumatology ·Vol. 19 ·No. 1 ·2001-00-00 ·Pages 103-6

Prieur AM

Abstract

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.

MeSH Terms
Age of Onset Arthritis, Juvenile/complications Arthropathy, Neurogenic/etiology Central Nervous System Diseases/congenital Chronic Disease Exanthema/etiology Humans Infant Infant Welfare Infant, Newborn Inflammation/complications Sex Factors Syndrome
Authors & Affiliations
1 authors, click to expand affiliations / ORCID
Prieur A M
Pediatric Immunology, Hematology and Rheumatology Unit, Hôpital Necker-Enfants Malades, AP-HP, University Paris V, Rue de Sèvres no. 149, F-75743 Paris, France.
Article Info
Journal
Clinical and experimental rheumatology
Abbr.
Clin Exp Rheumatol
ISSN
0392-856X
Published
2001-00-00
Pages
103-6
Language
English
Region
Italy
NLM ID
8308521
Subset
IM
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