Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease

F. S. van Leersum*, J. Potjewijd, M. van Geel, P. M. Steijlen, M. Vreeburg

*Corresponding author for this work

Research output: Contribution to journalComment/Letter to the editorAcademicpeer-review

7 Citations (Web of Science)


Schnitzler's syndrome is an auto-inflammatory disorder which is characterized by two mandatory features: an urticarial rash and a monoclonal gammopathy. Although the pathophysiology of this syndrome is not yet fully understood, a role for interleukin-1 seems apparent. While this presumed link between interleukin-1 and the monoclonal gammopathy is not yet elucidated, a mutual factor in pathophysiology however seems likely. Here we present a novel hypothesis of a shared pathophysiologic mechanism between Schitzler's syndrome and monoclonal gammopathy.

Original languageEnglish
Article number151
Number of pages4
JournalOrphanet Journal of Rare Diseases
Publication statusPublished - 22 Jun 2019


  • Schnitzler's syndrome
  • Waldenstroms macroglobulinemia
  • Autoinflammatory disease
  • Hypothesis
  • Interleukin-1
  • MYD88
  • NLRP3
  • IGM

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