Article Text

Beneficial response to anakinra and thalidomide in Schnitzler’s syndrome
  1. H D de Koning,
  2. E J Bodar,
  3. A Simon,
  4. J C H van der Hilst,
  5. M G Netea,
  6. J W M van der Meer
  1. Department of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  1. Correspondence to:
    Dr A Simon
    Radboud University Nijmegen Medical Centre, Department of General Internal Medicine, 484, PO Box 9101, 6500 HB Nijmegen, The Netherlands; a.simon{at}


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.

Case reports: 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.

Conclusion: 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.

  • CAPS, cryopyrin associated periodic syndrome
  • CRP, C reactive protein
  • IL1Ra, interleukin 1 receptor antagonist
  • SC, subcutaneously
  • Schnitzler’s syndrome
  • anakinra
  • thalidomide
  • interleukin 1
  • urticaria

Statistics from


  • Published Online First 11 August 2005

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.