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Tailoring biological treatment: anakinra treatment of posterior uveitis associated with the CINCA syndrome
  1. S C B Teoh1,
  2. S Sharma2,
  3. A Hogan2,
  4. R Lee3,
  5. A V Ramanan4,
  6. A D Dick5
  1. 1Bristol Eye Hospital, Bristol, UK
  2. 2Bristol Eye Hospital, Bristol, UK
  3. 3Department of Clinical Sciences at South Bristol, University of Bristol, Bristol Eye Hospital, Bristol, UK
  4. 4Department of Paediatric Rheumatology, North Bristol NHS Trust and Royal National Hospital for Rheumatic Diseases, Bath, UK
  5. 5Department of Clinical Sciences at South Bristol, University of Bristol, Bristol Eye Hospital, Bristol, UK
  1. Correspondence to: Professor A D Dick Department of Clinical Sciences at South Bristol, University of Bristol, Bristol Eye Hospital, Lower Maudlin Street, Bristol BS1 2LX, UK; a.dick{at}bristol.ac.uk

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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 …

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