SELECTED ARTICLES FROM THE RECENT LITERATURE 2007

August 13, 2007

The Schnitzler syndrome

Summary
Background – The Schnitzler syndrome (SS) is a rare disorder characterized by a chronic urticarial rash, intermittent fever, joint and bone pain, lymphodenopathy and the presence of a monoclonal gammopathy. The long-term course, including responses to therapy, is not well described.

Findings – deKonig et al of the Nijmegen Medical Center in the Netherlands investigated the clinical course of the SS by locating previous reports in the literature of 94 patients with the SS. They then contacted the authors of these reports with a questionnaire about their subsequent course of their SS patients.

In information was obtained in 43 of the SS patients followed for an average of 9.5 years. There has been no excessive age-related mortality in these 43 patients. However there have been no spontaneous remissions, either. There was a 15% risk of developing a distinct lymphoproliferative disorder over a 10 year period. There was a complete remission of the SS in all 8 patients treated with anti-interleukin-1 therapy.

Reference
Arthritis Rheum 2007;June 20, E pub

Editor's Comments
Although the SS appears to be rare, it should be considered in the differential diagnoses of individuals with chronic urticaria accompanied by fevers and bone/joint pain. An appropriate serum protein electrophoresis may make the diagnosis. The SS appear to be an auto-inflammatory disorder with the tissue target not yet identified.

 

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