Use of Anakinra (Kineret) in the Treatment of Familial Cold Autoinflammatory Syndrome with a 16-Month Follow-Up

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Abstract
Background: The susceptibility gene for familial cold autoinflammatory syndrome (FCAS) has been mapped to chromosome 1q44 and a point mutation determined to be present in all affected members of a large Canadian kindred. Anakinra (Kineret) is known to block IL-1 receptor and in the few patients with FCAS in whom it has been used, it has been shown to provide relief for this lifelong disability. Objective: To demonstrate the efficacy and safety of anakinra (Kineret) in FCAS. Methods: Eight affected family members aged 29 to 77 years received anakinra 100 mg subcutaneously daily for 4 weeks preceded and followed by a 2-week control period. Results: The treatment was rapidly effective paralleled by the immediate fall of the C-reactive protein and serum amyloid A protein. The only significant side effect was an injection-site reaction in 50%, which declined in the follow-up period. The effect was sustained in all who continued to use the treatment at 4 and 16 months of follow-up. Conclusion: This is the first treatment of FCAS that is completely effective while it is used.

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