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    Joint Bone Spine. 2008 May;75(3):263-6. Epub 2008 Feb 22.

    Schnitzler syndrome.

    Soubrier M.

    Gabriel Montpied Teaching Hospital, Service de Rhumatologie, 58 rue Montalembert, BP 69, 63003 Clermont-Ferrand Cedex 1, France. msoubrier@chu-clermontferrand.fr

    Schnitzler syndrome is characterized by monoclonal IgM gammopathy, urticaria, recurrent fever, evidence of inflammation, bone pain, and arthralgia, occasionally in combination with lymphadenopathy and/or hepatosplenomegaly. Only about 80 cases have been reported to date. Development of a hematological malignancy is the main complication. Recent reports of remissions induced by IL-1 receptor antagonist therapy shed new light on the pathophysiology of the disease.

    PMID: 18378180 [PubMed - indexed for MEDLINE]

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