Schnitzler Syndrome

  • Heleen D. de Koning
  • Karoline Krause


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.


Schnitzler syndrome Autoinflammatory Urticaria Monoclonal gammopathy Interleukin-1 



Cryopyrin-associated periodic syndrome


Chronic non-bacterial osteomyelitis


C-reactive protein


Chronic spontaneous urticaria


Erythrocyte sedimentation rate






Monoclonal gammopathy of unknown significance


Myeloid-related protein


Neutrophilic urticarial dermatosis


Peripheral blood mononuclear cells


Schnitzler syndrome


Toll-like receptor


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Heleen D. de Koning
    • 1
  • Karoline Krause
    • 2
  1. 1.Mauritskliniek NijmegenNijmegenThe Netherlands
  2. 2.Charité—Universitätsmedizin BerlinBerlinGermany

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