Schnitzlerové syndrom : Diferenciální diagnostika, přehled léčebných možností a popis 5 případů léčených anakinrou
Title in English | Schnitzler's Syndrome : Differential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra |
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Authors | |
Year of publication | 2016 |
Type | Article in Periodical |
Magazine / Source | Vnitřní lékařství |
MU Faculty or unit | |
Citation | |
Field | Oncology and hematology |
Keywords | anakinra; auto-inflammatory diseases; canakinumab; fever of unknown origin; FUO; interleukin 1; cryopyrin-associated autoinflammatory syndrome (CAPS); monoclonal gammopathy; rilonacept; Schnitzler's Syndrome; Adult Still's disease |
Description | Schnitzler's syndrome is an acquired auto-inflammatory disease of still unclear origin. The Strasbourg criteria were adopted (non-infectious fever, chronic urticaria, changes in the bone structure, leukocytosis and higher values of inflammatory markers - CRP and presence of monoclonal immunoglobulin mostly of type IgM, very rarely of IgG) to establish this diagnosis. The first-choice therapy for this disease is the blocking of interleukin-1 effects. In practice, the interleukin-1 receptor antagonist, anakinra, is the most commonly used. Currently reports also appear of the use of other medicines blocking the effect of interleukin-1, namely canakinumab and rilonacept. We have been treating 5 patients with anakinra (108, 72, 33, 32 and 1 months) on a long-term basis. |
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