Beneficial response to anakinra and thalidomide in Schnitzler's syndrome

Heleen D de Koning ¹, Evelien J Bodar ¹, Anna Simon ^1*, Jeroen CH van der Hilst ¹, Mihai G Netea ¹ and Jos WM van der Meer ¹

¹ Radboud University Nijmegen Medical Centre, Netherlands

^* To whom correspondence should be addressed. E-mail: a.simon{at}aig.umcn.nl.

Accepted 5 August 2005

Abstract

Schnitzlers syndrome is an inflammatory disorder characterised by chronic urticarial rash and monoclonal gammopathy, accompanied by periodic fever, arthralgia or arthritis and bone pain. Etiology and treatment are still enigmatic.

Objective: to assess therapy with thalidomide and interleukin-1 receptor antagonist (IL-1ra) anakinra in Schnitzlers syndrome.

Cases: we describe three patients with Schnitzler's syndrome, 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 six to eighteen months, all patients are free of symptoms.

Conclusion: Anakinra proved to be effective in three patients with Schnitzler's syndrome. In terms of side effects, this treatment is preferable to thalidomide, which induced a complete remission in one of our patients.

Keywords: Schnitzler's syndrome, anakinra, interleukin-1, thalidomide, urticaria

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