Shared MYD88/NF-κB mechanism hypothesis

Van Leersum FS, et al.

Orphanet J Rare Dis 14(1):151 · 2019

Grade Cexpert opinionengOpen AccessVerified

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.

Key Findings

  • Proposed shared MYD88/NF-κB mechanism

Subject Classification

AnimalsHereditary Autoinflammatory DiseasesHumansInterleukin-1Myeloid Differentiation Factor 88NLR Family, Pyrin Domain-Containing 3 ProteinSchnitzler SyndromeAutoinflammatory diseaseHypothesisInterleukin-1MYD88NLRP3Schnitzler’s syndromeWaldenströms macroglobulinemia

Referenced in (2 diseases)

Validation history

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  1. passsource metadataJun 1, 2026, 04:40 PMby pubmed-api

    Stored DOI was incorrect; corrected to the PubMed-registered DOI (verified to resolve to this article).

    tools: pubmed.esummary, crossref.works

ID: pmid-31228950DOI: 10.1186/s13023-019-1117-2PMID: 31228950PMCID: PMC6589170