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Schnitzler-Syndrom

Schnitzler syndrome

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Zusammenfassung

Das Schnitzler-Syndrom ist eine seltene systemische entzündliche Erkrankung, die gekennzeichnet ist durch das gemeinsame Vorhandensein einer chronischen Urtikaria und einer monoklonalen (IgM-) Gammopathie in Kombination mit weiteren, fakultativ vorhandenen Krankheitssymptomen. Es handelt sich um eine noch junge Krankheitsentität, die in den letzten Jahren zunehmende Bekanntheit erlangt und auch durch die Verfügbarkeit der Interleukin(IL)-1-Blockade als effektiver Therapieoption eine zunehmende Aufmerksamkeit erfährt. Aktuelle Erkenntnisse lassen die Erkrankung als eine Sonderform innerhalb des Kreises autoinflammatorischer Syndrome erscheinen. Der Artikel gibt einen Überblick über das klinische Erscheinungsbild, den gegenwärtigen Wissenstand zur Pathophysiologie und verfügbare Therapieoptionen.

Abstract

Schnitzler syndrome is a rare systemic inflammatory disease characterized by the presence of chronic urticarial skin rash and a monoclonal immunoglobulin M (IgM) gammopathy, combined with further, variable disease symptoms. The term refers to a young disease entity which has recently gained increasing acknowledgement and attention, also due to the availability of interleukin-1 (IL-1) blockade as an effective therapeutic option. Insights into the pathophysiology of the disease have resulted in the assumption of Schnitzler syndrome being a special form of an autoinflammatory disease with late onset or an acquired genesis. This article provides an overview on the clinical appearance, current knowledge of pathophysiology and available therapeutic options.

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Loock, J. Schnitzler-Syndrom. Z. Rheumatol. 71, 181–186 (2012). https://doi.org/10.1007/s00393-011-0857-8

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