The CINCA syndrome: a rare cause of chronic arthritis and multisystem inflammatory disorders.

Published online: Dec 27 2000

H de Boeck, T Scheerlinck, and J Otten.

Department of Pediatric, Orthopaedics A.Z.-V.U.B., Laarbeeklaan 101, B-1090 Brussels, Belgium.


Chronic infantile neurological cutaneous articular (CINCA) syndrome is a rare disorder with neonatal onset characterised by a chronic progressive inflammatory process with skin rash, articular and central nervous system involvement. This primary systemic inflammatory disorder should be distinguished from juvenile rheumatoid arthritis (JRA). Although the articular findings are characteristic features of CINCA syndrome, there is a certain degree of variability in the articular involvements which are not always symmetrical nor is the degree of severity uniform. The etiology of CINCA syndrome remains unknown. No single treatment has been found to be effective. This syndrome is known in the American medical literature as infantile onset multisystem inflammatory disease (IOMID).