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


CONCLUSIONS
This is the first treatment of FCAS that is completely effective while it is used.
Ross
J Barrie
JB
Division of Dermatology and Department of Medicine, Dalhousie University, Halifax, NS, Canada. jbross@dal.ca
Finlayson
Laura A
LA
Klotz
P Jennifer
PJ
Langley
Richard G
RG
Gaudet
Roxanne
R
Thompson
Kara
K
Churchman
Sarah M
SM
McDermott
Michael F
MF
Hawkins
Philip N
PN
D000328:Adult; D000368:Aged; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000453:epidemiology / Q000235:genetics; D002170:Canada / Q000453:epidemiology; D003080:Cold Temperature / Q000009:adverse effects*; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006801:Humans; D007249:Inflammation; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D013577:Syndrome; D016896:Treatment Outcome; D014581:Urticaria / Q000188:drug therapy* / Q000453:epidemiology / Q000235:genetics
D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein