Schnitzlerové syndrom : Diferenciální diagnostika, přehled léčebných možností a popis 5 případů léčených anakinrou

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Title in English Schnitzler's Syndrome : Differential diagnostics, an overview of therapeutic options and description of 5 cases treated with anakinra
Authors

ADAM Zdeněk ŠEDIVÁ Anna KOUKALOVÁ Renata ŘEHÁK Zdeněk PETRÁŠOVÁ Hana SZTURZ Petr ADAMOVÁ Zdenka VETEŠNÍKOVÁ Eva POUR Luděk KREJČÍ Marta SANDECKÁ Viera POUROVÁ Eva ČERMÁKOVÁ Zdeňka ŠEVČÍKOVÁ Sabina KRÁL Zdeněk MAYER Jiří

Year of publication 2016
Type Article in Periodical
Magazine / Source Vnitřní lékařství
MU Faculty or unit

Faculty of Medicine

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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