Anakinra: an effective treatment in the Schnitzler syndrome

Schnitzler syndrome is a rare disease characterized by chronic urticarial eruption and a monoclonal gammapathy. The exact pathogenesis is still unclear and treatment remains a challenge. We report the case of a patient with a 2-year history of arthralgia, chronic urticaria rash and fever, not responding to different treatments including anti-histaminics, corticosteroids, colchicine and low dose methotrexate. A diagnostic of Schnitzler syndrome was suggested and treatment with anakinra, an interleukin-1 receptor antagonist, was started, leading to a rapid, complete and sustained remission of symptoms. Anakinra seems to constitute an efficient and safe therapeutic approach for this rare disease.