Abstract
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 language | English |
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Article number | 151 |
Number of pages | 4 |
Journal | Orphanet Journal of Rare Diseases |
Volume | 14 |
Issue number | 1 |
DOIs | |
Publication status | Published - 22 Jun 2019 |
Keywords
- Schnitzler's syndrome
- Waldenstroms macroglobulinemia
- Autoinflammatory disease
- Hypothesis
- Interleukin-1
- MYD88
- NLRP3
- FOLLOW-UP
- IGM