Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2006 Apr;65(4):542-4.
doi: 10.1136/ard.2005.045245. Epub 2005 Aug 11.

Beneficial response to anakinra and thalidomide in Schnitzler's syndrome

H D de Koning  1 , E J Bodar, A Simon, J C H van der Hilst, M G Netea, J W M van der Meer
Affiliations
Case Reports

Beneficial response to anakinra and thalidomide in Schnitzler's syndrome

H D de Koning et al. Ann Rheum Dis. 2006 Apr.

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.

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.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. The Schnitzler syndrome. Four new cases and review of the literature. Medicine (Baltimore) 20018037–44. - PubMed
    1. Schnitzler L, Hurez D, Verret J L. Chronic urticaria ‐ hyperostosis ‐ macroglobulinemia. Ann Dermatol Venereol 1989116547–550. - PubMed
    1. Almerigogna F, Giudizi M G, Cappelli F, Romagnani S. Schnitzler's syndrome: what's new? J Eur Acad Dermatol Venereol 200216214–219. - PubMed
    1. Hawkins P N, Lachmann H J, Aganna E, McDermott M F. Spectrum of clinical features in Muckle‐Wells syndrome and response to anakinra. Arthritis Rheum 200450607–612. - PubMed
    1. Akimoto R, Yoshida M, Matsuda R, Miyasaka K, Itoh M. Schnitzler's syndrome with IgG kappa gammopathy. J Dermatol 200229735–738. - PubMed

Publication types