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J Cutan Med Surg. 2008 Jan-Feb;12(1):8-16.

Use of anakinra (Kineret) in the treatment of familial cold autoinflammatory syndrome with a 16-month follow-up.

Author information

1
Division of Dermatology and Department of Medicine, Dalhousie University, Halifax, NS, Canada. jbross@dal.ca

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.

PMID:
18258152
DOI:
10.2310/7750.2008.07050
[Indexed for MEDLINE]

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