title: Beneficial response to anakinra and thalidomide in Schnitzler's syndrome.
abstract: BACKGROUND
  Schnitzler's syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis, and bone pain. The cause and treatment are still unknown.
  
  
  OBJECTIVE
  To assess treatment with thalidomide and an interleukin 1 receptor antagonist, anakinra, in Schnitzler's syndrome.
  
  
  METHODS
  Three patients with Schnitzler's syndrome are described, one with IgM gammopathy, two with IgG type. In one patient, thalidomide induced complete remission, but was stopped because of polyneuropathy. Anakinra 100 mg daily in all three patients led to disappearance of fever and skin lesions within 24 hours. After a follow up of 6-18 months, all patients are free of symptoms.
  
  
  CONCLUSIONS
  Anakinra proved to be effective in three patients with Schnitzler's syndrome. This treatment is preferable to thalidomide, which induced a complete remission in one of our patients, as it has fewer side effects.
authors.affiliation: Radboud University Nijmegen Medical Centre, Department of General Internal Medicine, 6500 HB Nijmegen, The Netherlands.
authors.forename: H D
authors.initials: HD
authors.lastname: de Koning
authors.forename: E J
authors.initials: EJ
authors.lastname: Bodar
authors.forename: A
authors.initials: A
authors.lastname: Simon
authors.forename: J C H
authors.initials: JC
authors.lastname: van der Hilst
authors.forename: M G
authors.initials: MG
authors.lastname: Netea
authors.forename: J W M
authors.initials: JW
authors.lastname: van der Meer
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein; D008297:Male; D008875:Middle Aged; D017472:Receptors, Interleukin-1 / Q000037:antagonists & inhibitors; D019873:Schnitzler Syndrome / Q000188:drug therapy* / Q000473:pathology; D012795:Sialoglycoproteins / Q000627:therapeutic use*; D013792:Thalidomide / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; C507763:IL1RN protein, human; D053590:Interleukin 1 Receptor Antagonist Protein; D017472:Receptors, Interleukin-1; D012795:Sialoglycoproteins; D013792:Thalidomide