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Schnitzler Syndrome: a Review

  • L. Gusdorf
  • D. LipskerEmail author
Orphan Diseases (B Manger, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Orphan Diseases

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.

Keywords

Schnitzler syndrome Interleukin-1 Autoinflammation IL-1 blocking therapy Neutrophilic urticarial dermatosis Anakinra 

Notes

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Recently published papers of particular interest have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC 2017

Authors and Affiliations

  1. 1.Faculté de Médecine, Université de Strasbourg et Clinique DermatologiqueHôpitaux Universitaires de StrasbourgStrasbourgFrance

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