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Successful treatment of acute visual loss in Muckle-Wells syndrome with interleukin 1 receptor antagonist
  1. T Alexander1,
  2. O Klotz2,
  3. E Feist1,
  4. K Rüther2,
  5. G-R Burmester1,
  6. U Pleyer2
  1. 1Department of Rheumatology and Clinical Immunology, University School of Medicine Berlin, Germany
  2. 2Department of Ophthalmology Charité, University School of Medicine Berlin, Germany
  1. Correspondence to:
    MrT Alexander
    Charité University School of Medicine Berlin, Schumannstr 20/21, D-10117 Berlin, Germany; tobias.alexandercharite.de

https://doi.org/10.1136/ard.2004.032060

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    Muckle-Wells syndrome (MWS) is a dominantly inherited autoinflammatory disease characterised by urticarial skin rash, intermittent fever, arthralgia, and the frequent development of systemic AA amyloidosis. It is caused by mutations in the gene known as NALP3 or CIAS, which encodes a member of the purine superfamily of death domain fold proteins that are implicated in the regulation of inflammation through activation of nuclear factor κB (NF-κB) and regulation of interleukin 1 (IL1) processing.1,2 Over recent years, therapeutic trials with the human recombinant IL1 receptor antagonist anakinra have reported encouraging results in patients with various autoinflammatory syndromes.3,4 We report here the unusual case of acute visual loss in a patient with MWS and its good response to anakinra.

    CASE REPORT

    A 70 year old woman with a history of …

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