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.
Keywords
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- 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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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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