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Schnitzler syndrome

GtoPdb Disease Summaries

This section gives an overview of the disease, and where available shows the following:

  • Synonyms: Shows known synonyms for the disease.
  • Description: Gives a basic description/definition of the disease.
  • Database Link: External links to the same disease at the Disease Ontology, OMIM or Orphanet sites.
  • Immunopharmacology comments: General comments about the target's role in immunopharmacology, provided by GtoImmuPdb curators.
  • Associated with: Counts are displayed for the total targets the disease is associated with in GtoPdb. The counts of targets and ligands of immunological relevance associated to the disease are also shown.

More information can be found in the help pages.

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Disease ID:1258
Name:Schnitzler syndrome
Associated with:0 target
1 immuno-relevant ligand
Synonyms
Chronic urticaria with gammopathy | Chronic urticaria with macroglobulinemia
Description
A rare autoinflammatory disorder that is characterised by late-onset of chronic hives (urticaria), periodic fever, bone and/or joint pain and/or inflammation, weight loss, malaise, fatigue, leukocytosis and systemic inflammatory response including enlarged spleen and liver.
Database Links
Orphanet: ORPHA37748

Targets

GtoPdb Disease Summaries - Targets

Click on the target name to link to its detailed view page

Where available, information is display on the role of the target in the disease; drugs which target the disease and their therapeutic use and side-effects.

If there is mutation data curated in GtoPdb this is indicated, with a link back to the appropriate section on the target detailed view page

Immuno ligand interactions - If available, a table of immuno-relevant ligands is shown. These ligands have been curated as having an association to the disease and possess interaction data with the target in GtoPdb. The approval status of the ligand is shown, along with curator comments and an indication of whether the target is considered the primary target of the ligand.

More information can be found in the help pages.

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Key to terms and symbols

No target related data available for Schnitzler syndrome

Ligands

GtoPdb Disease Summaries - Ligands

Click ligand name to view ligand summary page

  • Approved: If the ligand is an approved drug this is indicated, along with approval bodies.
  • Immuno: Immuno icon indicates the ligand is immuno-relevant

Click the arrow in the final column to expand comments

  • Immuno Disease Comments: Curatorial comments specifically added as part of GtoImmuPdb. They give more information on the association between the ligand and disease in the context of immunopharmacology.
  • Clinical Use: General clinical comments relating to the ligand and may not necessarily be specific to the disease in question. With hyperlink to more details on the ligand summary pages.
  • Bioactivty Comments: Curatorial comments specifically about the compounds biological activity - with hyperlink to more details on the ligand summary pages.

More information can be found in the help pages.

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Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
dapansutrile
Immuno Disease Comments: Phase 1 clinical candidate for Schnitzler syndrome- see NCT03595371.
Clinical Use: Clinical trials using dapansutrile (OLT1177) in acute and chronic inflammatory conditions were initiated, to evaluate both topical and oral efficacy [2,4]. The only active study listed on ClinicalTrials.org in Feb. 2024 was in participants with gouty arthritis. There are also plans to assess dapansutrile to tackle neuroinflammatory pathology in Parkinson's disease [3] based on evidence from preclinical animal models [1].

SARS-CoV-2 and COVID-19: Dapansutrile's anti-inflammatory potential (via oral administration) was planned to be investigated in COVID-19 patients with moderate symptoms, but that clinical trial (NCT04540120) was terminated in response to the successful deployment of anti-SARS-CoV-2 vaccines. | View clinical data

References

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1. Amo-Aparicio J, Daly J, Højen JF, Dinarello CA. (2023) Pharmacologic inhibition of NLRP3 reduces the levels of α-synuclein and protects dopaminergic neurons in a model of Parkinson's disease. J Neuroinflammation, 20 (1): 147. [PMID:37349821]

2. Klück V, Jansen TLTA, Janssen M, Comarniceanu A, Efdé M, Tengesdal IW, Schraa K, Cleophas MCP, Scribner CL, Skouras DB et al.. (2020) Dapansutrile, an oral selective NLRP3 inflammasome inhibitor, for treatment of gout flares: an open-label, dose-adaptive, proof-of-concept, phase 2a trial. Lancet Rheumatol, 2 (5): e270-e280. [PMID:33005902]

3. Stefanova N, Dinarello CA, Amo-Aparicio J. Assessing the Ability of Dapansutrile, a Selective, Oral Inhibitor of the NLRP3 Neuroinflammatory Pathway, to Mitigate Progression of Parkinson’s Disease. Accessed on 22/02/2024. Modified on 22/02/2024. michaeljfox.org, https://www.michaeljfox.org/grant/assessing-ability-dapansutrile-selective-oral-inhibitor-nlrp3-neuroinflammatory-pathway

4. Zhou W, Chen C, Chen Z, Liu L, Jiang J, Wu Z, Zhao M, Chen Y. (2018) NLRP3: A Novel Mediator in Cardiovascular Disease. J Immunol Res, 2018: 5702103. [PMID:29850631]