title: Effect of etanercept and anakinra on inflammatory attacks in the hyper-IgD syndrome: introducing a vaccination provocation model.
abstract: BACKGROUND
  Hyper-IgD and periodic fever syndrome (HIDS) is an hereditary autoinflammatory syndrome, characterised by recurrent inflammatory attacks. Treatment of HIDS is difficult, although simvastatin is beneficial and etanercept might be effective. Studying the treatment of a rare periodic syndrome is complicated by the varying frequency and severity of symptoms and low prevalence. Our aim was to develop a system of clinical observations to evaluate effectiveness of treatment-on-demand.
  
  
  METHODS
  Seven fever episodes in three HIDS patients were monitored, with and without administration of etanercept or anakinra. We developed a clinical score, which includes 12 symptoms. In one patient, inflammatory attacks were provoked by vaccination.
  
  
  CONCLUSIONS
  At the onset of an attack, all patients reported a clinical score between 20 and 25. The score was used to quantify severity and define the end of an attack. Reproducible monitoring of inflammatory episodes was difficult, even in this pilot study. The effect of early administration of etanercept was variable. In one patient, a fever episode could be readily provoked within 12 to 24 hours by vaccination. In this patient, the IL-1ra analogue anakinra was more successful in aborting the inflammatory attack than etanercept. We propose that this vaccination model will allow evaluation of treatment-on-demand in a controlled setting.
authors.affiliation: Division of General Internal Medicine, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
authors.forename: E J
authors.initials: EJ
authors.lastname: Bodar
authors.forename: J C H
authors.initials: JC
authors.lastname: van der Hilst
authors.forename: J P H
authors.initials: JP
authors.lastname: Drenth
authors.forename: J W M
authors.initials: JW
authors.lastname: van der Meer
authors.forename: A
authors.initials: A
authors.lastname: Simon
mesh_terms: D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage / Q000627:therapeutic use*; D002097:C-Reactive Protein / Q000378:metabolism; D000068800:Etanercept; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000139:chemically induced / Q000188:drug therapy*; D007072:Immunoglobulin D; D007074:Immunoglobulin G / Q000008:administration & dosage / Q000627:therapeutic use*; D007279:Injections, Subcutaneous; D053590:Interleukin 1 Receptor Antagonist Protein; D018124:Receptors, Tumor Necrosis Factor / Q000008:administration & dosage / Q000627:therapeutic use*; D012795:Sialoglycoproteins / Q000008:administration & dosage / Q000627:therapeutic use*; D014612:Vaccines / Q000009:adverse effects*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; C507763:IL1RN protein, human; D007072:Immunoglobulin D; D007074:Immunoglobulin G; D053590:Interleukin 1 Receptor Antagonist Protein; D018124:Receptors, Tumor Necrosis Factor; D012795:Sialoglycoproteins; D014612:Vaccines; D002097:C-Reactive Protein; D000068800:Etanercept