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. (C) 2011 Societe francaise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.