Management of Schnitzler's syndrome

Introduction: Schnitzler's syndrome is a paradigm of an acquired autoinflammatory disorder, involving IL-1, which can now be pharmacologically targeted. Areas covered: Management of Schnitzler's syndrome is covered in detail, by reviewing all published papers on this topic until May 2014. Schnitzler's syndrome usually affects adult patients in their fifties. The main symptoms are an urticarial rash, fever, joint and/or bone pain, elevated CRP and leukocytosis in a patient with a monoclonal IgM (or rarely IgG) gammopathy. It is a chronic/recurrent disorder and spontaneous remissions are exceedingly rare. Patients are at specific risk of inflammatory anemia and AA-amyloidosis. The risk of associated lymphoid malignancy seems to be comparable to the one associated to monoclonal gammopathy of undetermined significance in general. Diagnosis relies on the Lipsker or the Strasbourg criteria. Expert opinion: Treatment relies on IL-1 inhibitors which allow a rapid and complete control of all signs. The IL-1 receptor antagonist anakinra, which has a short half-life and necessitates daily injections, is the treatment of choice in patients with Schnitzler's syndrome who have alteration in their quality of life or persistent elevation of markers of inflammation. The other IL-1 inhibitors with longer half-lives need further investigation.