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Current Rheumatology Reports
Published in: Current Rheumatology Reports 8/2017

01-08-2017 | Orphan Diseases (B Manger, Section Editor)

Schnitzler Syndrome: a Review

Authors: L. Gusdorf, D. Lipsker

Published in: Current Rheumatology Reports | Issue 8/2017

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Abstract

Purpose of Review

We focus on recent advances in diagnosis and therapeutic strategies, as well as on pathogenesis of Schnitzler syndrome.

Recent Findings

New diagnostic criteria were established, and their external validity was assessed in a retrospective cohort study. The cytokine interleukin-1 (IL-1) plays a crucial role in the pathogenesis of the Schnitzler syndrome, and this explains the spectacular efficiency of IL-1 blocking therapies.

Summary

The Schnitzler syndrome is now considered as a late-onset acquired autoinflammatory syndrome in which the cytokine IL-1 plays a crucial role. IL-1 blocking therapies are efficient on the inflammation-linked symptoms but not on the monoclonal component. Therefore, they probably don’t reduce the risk of the development of lymphoproliferative disorders that remains the main prognostic issue. The link between autoinflammation and the monoclonal component needs to be further elucidated.
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Metadata
Title
Schnitzler Syndrome: a Review
Authors
L. Gusdorf
D. Lipsker
Publication date
01-08-2017
Publisher
Springer US
Published in
Current Rheumatology Reports / Issue 8/2017
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI
https://doi.org/10.1007/s11926-017-0673-5

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