Schnitzler-syndrom – paradigma multidisciplinárního přístupu u velmi vzácné diagnózy

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Title in English Schnitzler syndrome a paradigm of multidisciplinary approach in a very rare diagnosis
Authors

SZTURZ Petr ŠTORK Jiří ČERMÁKOVÁ Zdeňka GOTTWALDOVÁ Jana ŠEDIVÁ Anna ADAM Zdeněk FOJTÍK Zdeněk STEYEROVÁ Petra VOKÁČOVÁ Alena KOUKALOVÁ Renata ŘEHÁK Zdeněk MAYER Jiří

Year of publication 2014
Type Article in Periodical
Magazine / Source Postgraduální medicína
MU Faculty or unit

Faculty of Medicine

Citation
Field Oncology and hematology
Keywords Schnitzler syndrome; monoclonal immunoglobulin; cytokines; interleukins; anakinra; biologic medication
Attached files
Description Schnitzler syndrome is a very rare idiopathic disease characterized above all by: chronic urticaria, monoclonal gammopathy (immunoglobulin M or G types) and proinflammatory state of the organism. Moreover, it should be considered in differential diagnosis of fever of unknown origin, chronic disease anemia, pains of the musculoskeletal system as well as lymphadenopathy. Due to recurring nature of inflammatory response of the organism, predisposition for excessive stimulated interleukin-1beta secretion and certain similarities with cryopyrinopathies (neutrophilic urticarial dermatosis in skin biopsy, almost universal treatment efficacy of interleukin-1 receptor antagonist – anakinra, perhaps also NLRP3 gene mutation), Schnitzler syndrome is being increasingly classified as an acquired autoinflammatory disorder. In our work, we stress a multidisciplinary approach to Schnitzler syndrome from dermatologist’s, hematologist’s, oncologist’s, internist’s, rheumatologist’s, immunologist’s and pathophysiologist’s points of view. Regarding potentially life-threatening complications however (risks of transformation into a lymphoproliferation and development of secondary amyloidosis), we aim to address as many medical specialties as possible. With this objective in mind, we have added to the article an illustrated supplementary material including clinical findings, results from imaging methods and laboratory tests as well as a histopathological examination of a skin biopsy.
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