Schnitzler Syndrome Is Effectively Managed with Long-Term Administration of Anakinra

Schnitzler syndrome is the combination of urticarial skin lesions with monoclonal gammopathy, primarily IgM, and other systemic manifestations, including fever, bone and joint pain, osteosclerotic bone lesions, lymphadenopathy, and hepatosplenomegaly. Some patients progress to develop hematologic malignancies. Various biologic agents have been reported to be effective, among them the interleukin 1 receptor antagonist anakinra.

These authors report their experience with long-term use of anakinra in patients with Schnitzler syndrome. They retrospectively reviewed the cases of 42 patients from multiple French institutions, 29 of whom had received anakinra therapy; 25 were in complete remission with therapy, and 5 were in partial remission. A st…