| Summary: | Schnitzler syndrome is a rare inflammatory disorder characterized by chronic urticarial rash, monoclonal gammopathy, periodic fever, arthralgia/arthritis and bone pain. However, the results of management of Schnitzler syndrome are often disappointing and its treatment remains a challenge. No cases of spontaneous complete remission have been reported. Anakinra is an interleukin 1 receptor antagonist used for the treatment of rheumatoid arthritis, and has been reported to be universally effective for Schnitzler syndrome. Here, we report the first Asian patient with Schnitzler syndrome who achieved long-term control with anakinra, after previous failures with various therapeutic approaches.
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