title: Use of anakinra (Kineret) in the treatment of familial cold autoinflammatory syndrome with a 16-month follow-up.
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.
  
  
  CONCLUSIONS
  This is the first treatment of FCAS that is completely effective while it is used.
authors.affiliation: Division of Dermatology and Department of Medicine, Dalhousie University, Halifax, NS, Canada. jbross@dal.ca
authors.forename: J Barrie
authors.initials: JB
authors.lastname: Ross
authors.forename: Laura A
authors.initials: LA
authors.lastname: Finlayson
authors.forename: P Jennifer
authors.initials: PJ
authors.lastname: Klotz
authors.forename: Richard G
authors.initials: RG
authors.lastname: Langley
authors.forename: Roxanne
authors.initials: R
authors.lastname: Gaudet
authors.forename: Kara
authors.initials: K
authors.lastname: Thompson
authors.forename: Sarah M
authors.initials: SM
authors.lastname: Churchman
authors.forename: Michael F
authors.initials: MF
authors.lastname: McDermott
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
mesh_terms: 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
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein