CONCISE REPORT

Beneficial response to anakinra and thalidomide in Schnitzler’s syndrome

H D de Koning, E J Bodar, A Simon, J C H van der Hilst, M G Netea, J W M van der Meer

Department of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands

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}aig.umcn.nl

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.

Abbreviations: CAPS, cryopyrin associated periodic syndrome; CRP, C reactive protein; IL1Ra, interleukin 1 receptor antagonist; SC, subcutaneously

Keywords: Schnitzler’s syndrome; anakinra; thalidomide; interleukin 1; urticaria

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Pizzirani, C., Falzoni, S., Govoni, M., Corte, R. L., Donadei, S., Virgilio, F. D., Trotta, F., Monaco, A. L. (2009). Dysfunctional inflammasome in Schnitzler's syndrome. Rheumatology (Oxford) 48: 1304-1308 [Abstract] [Full Text]  
• van Deuren, M., Kroot, J. J. C., Swinkels, D. W. (2009). Time-course analysis of serum hepcidin, iron and cytokines in a C282Y homozygous patient with Schnitzler's syndrome treated with IL-1 receptor antagonist. haematol 94: 1297-1300 [Abstract] [Full Text]  
• LEQUERRE, T., VITTECOQ, O., LE LOET, X. (2009). Dr. Lequerre, et al reply. The Journal of Rheumatology 36: 447-447 [Full Text]  
• de Koning, H. D., van der Meer, J. W. M., Simon, A. (2007). Comment on: Schnitzlers syndrome exacerbation after anti-TNF treatment. Rheumatology (Oxford) 46: 1741-1741 [Full Text]  
• Asli, B., Bienvenu, B., Cordoliani, F., Brouet, J.-C., Uzunhan, Y., Arnulf, B., Malphettes, M., Rybojad, M., Fermand, J.-P. (2007). Chronic Urticaria and Monoclonal IgM Gammopathy (Schnitzler Syndrome): Report of 11 Cases Treated With Pefloxacin. Arch Dermatol 143: 1046-1050 [Abstract] [Full Text]  
• Thonhofer, R., Uitz, E., Graninger, W. (2007). Schnitzler's syndrome--exacerbation after anti-TNF treatment. Rheumatology (Oxford) 46: 1041-1042 [Full Text]