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CASE REPORTS
JOURNAL ARTICLE
REVIEW
Schnitzler syndrome: response to anakinra in two cases and a review of the literature.
International Journal of Dermatology 2009 November
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.
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.
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