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Schnitzler Syndrome: a Review.

L Gusdorf¹, D Lipsker²

¹Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Current Rheumatology Reports

|July 19, 2017

Summary

Schnitzler syndrome is an acquired autoinflammatory disorder. Interleukin-1 (IL-1) blockade treats inflammation but not the monoclonal component, leaving lymphoproliferative disorder risk unaddressed.

Keywords:

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

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Area of Science:

Rheumatology
Immunology
Hematology

Background:

Schnitzler syndrome is a rare autoinflammatory condition.
Recent advances have improved understanding of its pathogenesis and treatment.

Purpose of the Study:

To review recent advances in Schnitzler syndrome diagnosis and therapy.
To explore the pathogenesis of Schnitzler syndrome, focusing on the role of IL-1.

Main Methods:

Review of recent literature on Schnitzler syndrome.
Assessment of external validity of new diagnostic criteria using a retrospective cohort study.

Main Results:

New diagnostic criteria for Schnitzler syndrome have been established and validated.
Interleukin-1 (IL-1) is central to Schnitzler syndrome pathogenesis, explaining the efficacy of IL-1 blocking therapies.
IL-1 blockade effectively manages inflammation-related symptoms but does not impact the monoclonal component or reduce the risk of lymphoproliferative disorders.

Conclusions:

Schnitzler syndrome is a late-onset acquired autoinflammatory syndrome driven by IL-1.
While IL-1 blockade is effective for inflammatory symptoms, the link between autoinflammation and the monoclonal component requires further investigation.
The risk of lymphoproliferative disorders remains a significant prognostic concern, necessitating further research.