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Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: a French multicenter study

Antoine Néel, Benoit Henry, Sebastien Barbarot, Agathe Masseau, François Perrin, Claire Bernier, Xavier Kyndt, Xavier Puechal, Pierre-Jean Weiller, Olivier Decaux, Jacques Ninet, Arnaud Hot, Achille Aouba, Leonardo Astudillo, Jean-Marie Berthelot, Fabrice Bonnet, Jean-Marie Brisseau, Bérangère Cador, Fabienne Closs-Prophette, Thomas Dejoie, Jean-Dominique de Korwin, Robin Dhote, Renato Fior, Bernard Grosbois, Eric Hachulla, Pierre-Yves Hatron, Henry Jardel, David Launay, Adrien Lorleac'h, Pierre Pottier, Guillaume Moulis, Jacques Serratrice, Amar Smail, Mohamed Hamidou
Autoimmunity Reviews 2014, 13 (10): 1035-41
The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5years (range: 1.6-35). Two patients developed Waldenström's macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown.

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