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Schnitzler Syndrome

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Textbook of Autoinflammation

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Abstract

Schnitzler syndrome (SchS) is a late-onset autoinflammatory disease characterized by the association of a chronic urticarial rash and monoclonal gammopathy with signs and symptoms of systemic inflammation. Clinical efficacy of IL-1ß blocking drugs revealed the key role of IL-1ß in the pathophysiology of SchS. This was corroborated by in vitro and genetic studies. Anti-IL-1ß treatment abrogates the systemic inflammation, but leaves the monoclonal gammopathy unaffected. The role of the monoclonal gammopathy (cause or consequence) is the major question that remains to be resolved.

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Abbreviations

CAPS:

Cryopyrin-associated periodic syndrome

CNO:

Chronic non-bacterial osteomyelitis

CRP:

C-reactive protein

CSU:

Chronic spontaneous urticaria

ESR:

Erythrocyte sedimentation rate

Ig:

Immunoglobulin

IL-1:

Interleukin-1

MGUS:

Monoclonal gammopathy of unknown significance

MRP:

Myeloid-related protein

NUD:

Neutrophilic urticarial dermatosis

PBMCs:

Peripheral blood mononuclear cells

SchS:

Schnitzler syndrome

TLR:

Toll-like receptor

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Author information

Authors and Affiliations

  1. Mauritskliniek Nijmegen, Nijmegen, The Netherlands

    Heleen D. de Koning

  2. Charité—Universitätsmedizin Berlin, Berlin, Germany

    Karoline Krause

Authors
  1. Heleen D. de Koning
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  2. Karoline Krause
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Editor information

Editors and Affiliations

  1. Pediatric Rheumatology Unit, Shaare Zedek Medical Center and Hebrew University, Jerusalem, Israel

    Philip J. Hashkes

  2. Department Paediatrics, Division of Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada

    Ronald M. Laxer

  3. Department of Internal Medicine, Radboudumc Expertisecenter for Immunodeficiency and Autoinflammation, Radboud University Medical Center, Nijmegen, The Netherlands

    Anna Simon

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de Koning, H.D., Krause, K. (2019). Schnitzler Syndrome. In: Hashkes, P., Laxer, R., Simon, A. (eds) Textbook of Autoinflammation. Springer, Cham. https://doi.org/10.1007/978-3-319-98605-0_37

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  • DOI: https://doi.org/10.1007/978-3-319-98605-0_37

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-98604-3

  • Online ISBN: 978-3-319-98605-0

  • eBook Packages: MedicineMedicine (R0)

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