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Updated: May 22, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Schnitzler syndrome]
1Klinikum Bad Bramstedt, Bad Bramstedt, Deutschland. jloock@schoen-kliniken.de
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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