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Still's disease, adult onset

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:1262
Name:Still's disease, adult onset
Associated with:0 target
2 immuno-relevant ligands
Description
Adult onset Still's disease (AoSD) is a rare systemic inflammatory condition of unknown aetiology. It is characterised by episodes of high, spiking fevers, arthritic jint inflammation/pain and a salmon coloured rash. AoSD is the adult form of juvenile idiopathic arthritis (a.k.a. juvenile Still's disease). There is some evidence to suggest that AoSD is an autoinflammatory syndrome, that may or may not be potentiated by an abnormal or exaggerated immune response to an infection or toxin.
Database Links
Disease Ontology: DOID:14256

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 Still's disease, adult onset

Ligands

GtoPdb Disease Summaries - Ligands

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  • 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
tadekinig alfa 2
Immuno Disease Comments: Tadekinig alfa is an FDA orphan drug and Phase 2 clinical candidate for AoSD (see NCT02398435).
Clinical Use: Tadekinig alfa has advanced to Phase 3 clinical evaluation in patients with spontaneous activating mutations of the NLRC4 and with recurrent MAS-like flares driven by constitutive IL-18 hypersecretion. A further trial is evaluating tadekinig alfa in hemophagocytic lymphohistiocytosis patients with XIAP deficiency, which is a MAS-like syndrome that is characterised by high levels of serum IL-18. Favourable Phase 2 efficacy and safety of tadekinig alfa in patients with adult-onset Still's disease was reported in June 2018 [2]. | View clinical data
camoteskimab 3
Immuno Disease Comments: Orpahn drug (Feb. 2024) for the treatment of AOSD.
Clinical Use: The FDA granted orphan designation in February 2024, for the treatment of adult-onset Still's disease and systemic juvenile idiopathic arthritis [1]. | View clinical data

References

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1. FDA. Camoteskimab. Accessed on 26/02/2024. Modified on 26/02/2024. fda.gov, https://www.accessdata.fda.gov/scripts/opdlisting/oopd/detailedIndex.cfm?cfgridkey=961623

2. Gabay C, Fautrel B, Rech J, Spertini F, Feist E, Kötter I, Hachulla E, Morel J, Schaeverbeke T, Hamidou MA et al.. (2018) Open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alfa (IL-18BP) in adult-onset Still's disease. Ann Rheum Dis, 77 (6): 840-847. [PMID:29472362]

3. Galozzi P, Bindoli S, Doria A, Sfriso P. (2022) Progress in Biological Therapies for Adult-Onset Still's Disease. Biologics, 16: 21-34. [PMID:35481241]