Schnitzler Syndrome: a Review

@article{Gusdorf2017SchnitzlerSA,
  title={Schnitzler Syndrome: a Review},
  author={Laurence Gusdorf and Dan Lipsker},
  journal={Current Rheumatology Reports},
  year={2017},
  volume={19},
  pages={1-6}
}
Purpose of ReviewWe focus on recent advances in diagnosis and therapeutic strategies, as well as on pathogenesis of Schnitzler syndrome.Recent FindingsNew 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.SummaryThe Schnitzler syndrome is now considered as a… 
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TLDR
The IL-1 receptor antagonist anakinra, which has a short half-life and necessitates daily injections, is the treatment of choice in patients with Schnitzler’s syndrome who have alteration in their quality of life or persistent elevation of markers of inflammation.
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This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date of Schnitzler’s syndrome.
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TLDR
The Schnitzler syndrome resembles the genetically determined auto-inflammatory syndromes involving activating mutations of the NLRP3 inflammasome and its consequences will be addressed.
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TLDR
The bone scintigraphy pattern may be very suggestive of the correct diagnosis and of bone involvement in this syndrome and lead to a differential diagnosis including infiltrative diseases and dysplastic diseases.
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TLDR
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TLDR
The Schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy, and the disease pursues a chronic course, and no remissions have yet been reported.
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TLDR
First‐line treatment in patients with significant alteration of quality of life or persistent elevation of markers of inflammation should be anakinra and follow‐up should include clinical evaluation, CBC and CRP every 3 months and MGUS as usually recommended.
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