Dramatic improvement with anakinra in a case of chronic infantile neurological cutaneous and articular (CINCA) syndrome
Open Access
- 25 January 2005
- journal article
- research article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 44 (5) , 689-690
- https://doi.org/10.1093/rheumatology/keh547
Abstract
Sir, Chronic infantile neurological, cutaneous and articular (CINCA) syndrome, also known as neonatal-onset multisystem inflammatory disease (NOMID), is a rare genetic systemic autoinflammatory disease characterized by mutation in the CIAS1 (cold autoinflammatory syndrome-1) gene [1, 2]. Renal AA-amyloidosis, severe arthropathy and neurological complications are long-term disabilities with this disease. CINCA syndrome is poorly treatable and steroids do not completely eliminate the disease and can cause harmful side-effects. We report a case of CINCA syndrome which was dramatically improved with anakinra, an interleukin-1-receptor antagonist (IL1-RA).Keywords
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