BACKGROUND: Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation. The pathophysiology is still unknown, although various autoantibody-mediated mechanisms have been described. Complete remission of symptoms has been reported recently in patients with Schnitzler syndrome treated with anakinra, an interleukin-1 receptor antagonist. METHODS: Two patients with Schnitzler syndrome treated with anakinra therapy are presented. RESULTS: We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy. A review of the published literature on the treatment of Schnitzler syndrome with anakinra is presented. CONCLUSIONS: Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms.
BACKGROUND:Schnitzler syndrome is a rare disease characterized by a chronic urticarial eruption and monoclonal gammopathy, as well as clinical and laboratory signs of inflammation. The pathophysiology is still unknown, although various autoantibody-mediated mechanisms have been described. Complete remission of symptoms has been reported recently in patients with Schnitzler syndrome treated with anakinra, an interleukin-1 receptor antagonist. METHODS: Two patients with Schnitzler syndrome treated with anakinra therapy are presented. RESULTS: We report two cases of nearly complete remission of symptoms in Schnitzler syndrome after the initiation of anakinra therapy, and the first observation of a relapse under continuous daily anakinra therapy. A review of the published literature on the treatment of Schnitzler syndrome with anakinra is presented. CONCLUSIONS: Based on published data, monotherapy with anakinra is currently the most promising treatment for Schnitzler syndrome, because it is able to induce complete remission of symptoms.
Authors: Heleen D de Koning; Joost Schalkwijk; Monique Stoffels; Johanna Jongekrijg; Joannes F M Jacobs; Eugène Verwiel; Hans J P M Koenen; Frank Preijers; Dirk Holzinger; Irma Joosten; Jos W M van der Meer; Anna Simon Journal: Arthritis Res Ther Date: 2015-07-22 Impact factor: 5.156