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Chronic infantile neurological cutaneous articular (CINCA) syndrome, also known as neonatal-onset multisystem inflammatory disease, is a rare congenital inflammatory disease characterised by cardinal signs including a variable congenital maculopapular urticarial rash, chronic non-inflammatory arthropathy with abnormal cartilage proliferation, and chronic meningitis with progressive neurological impairment associated with polymorphonuclear and occasionally eosinophilic infiltration.1 The CINCA syndrome is associated with childhood uveitis and papillitis with chronic disc swelling.2 It may occur as a result of mutations of the CIAS1 gene that encodes cryopyrin, which results in reduced apoptosis of the inflammatory cells with up regulation of interleukin 1 (IL1).3–5 Consequently, the CINCA syndrome responds poorly to immunosuppressives including steroids, and treatment has been limited until recent reports of successful treatment with the recombinant human IL1 receptor antagonist (rHuIL1Ra), anakinra (Kineret, Amgen, Thousand Oaks, California).6,7,8,9,10 We report the case of successful treatment with rHuIL1Ra of a patient with refractory …
Competing interests: None declared.
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