We are analyzing https://link.springer.com/article/10.1186/s12969-015-0047-3.

Title:
Gene-expression analysis of adult-onset Still’s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity | Pediatric Rheumatology
Description:
Background Adult-onset Still’s disease (AOSD), a rare autoinflammatory disorder, resembles systemic juvenile idiopathic arthritis (SJIA). The superimposable systemic clinical features of AOSD and SJIA suggest both clinical phenotypes represent the same disease continuum with different ages of onset. To further characterize the similarity between AOSD and SJIA at the molecular level, 2 previously identified response gene sets in SJIA were used to investigate how genes that respond to interleukin (IL)-1β inhibition with canakinumab in SJIA patients behave in AOSD patients with active disease prior to IL-1β targeting therapy, relative to healthy subjects. Findings All genes downregulated in SJIA patients following canakinumab treatment were upregulated in most patients with active AOSD prior to canakinumab treatment, relative to healthy subjects. A few patients with milder AOSD had expectedly gene-expression patterns that resembled those in healthy subjects. Comparison of the gene-expression patterns with neutrophil counts showed a correlation between elevated neutrophil numbers and upregulation of canakinumab-responsive genes. Correspondingly, most genes upregulated following canakinumab treatment in patients with SJIA patients were downregulated in the majority of AOSD patients. Conclusions These results further support the concept of a Still’s disease continuum that includes both a pediatric/juvenile onset (SJIA) and adult onset (AOSD) form.
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Keywords {🔍}

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Topics {✒️}

il-1β long-acting inhibitor anti − il-1β therapy article download pdf gene-expression profiles christof specker expectedly gene-expression patterns previous gene-expression analysis il-1β targeting therapy anti − il1-β therapy present gene-expression analysis 1st-strand cdna synthesis 2nd-strand cdna synthesis il1-receptor antagonist anakinra received grants/research support pediatric/juvenile onset ribo-spia® amplification process single disease entity aosd gene-expression patterns full size image il-1–driven condition similar il-1r inhibitor anakinra gov/ct2/show/nct02204293 identify canakinumab-responsive genes gene-expression patterns macrophage activation syndrome blood cell counts author information authors privacy choices/manage cookies active systemic features probe sets identified gene-expression values innate-immunity–related genes gene expression profiling neutrophil counts showed gene-expression analysis elevated neutrophil numbers arthritis res ther ovation wb reagent creative commons license il-1β inhibition immune-cell activity semin arthritis rheum rare autoinflammatory disorder gene-expression data gene expression data il-1 − driven condition treatment-resistant adult-onset norbert blank innate immune system support research activities

Questions {❓}

Adult-onset Still’s disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy?

Schema {🗺️}

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         headline:Gene-expression analysis of adult-onset Still’s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity
         description:Adult-onset Still’s disease (AOSD), a rare autoinflammatory disorder, resembles systemic juvenile idiopathic arthritis (SJIA). The superimposable systemic clinical features of AOSD and SJIA suggest both clinical phenotypes represent the same disease continuum with different ages of onset. To further characterize the similarity between AOSD and SJIA at the molecular level, 2 previously identified response gene sets in SJIA were used to investigate how genes that respond to interleukin (IL)-1β inhibition with canakinumab in SJIA patients behave in AOSD patients with active disease prior to IL-1β targeting therapy, relative to healthy subjects. All genes downregulated in SJIA patients following canakinumab treatment were upregulated in most patients with active AOSD prior to canakinumab treatment, relative to healthy subjects. A few patients with milder AOSD had expectedly gene-expression patterns that resembled those in healthy subjects. Comparison of the gene-expression patterns with neutrophil counts showed a correlation between elevated neutrophil numbers and upregulation of canakinumab-responsive genes. Correspondingly, most genes upregulated following canakinumab treatment in patients with SJIA patients were downregulated in the majority of AOSD patients. These results further support the concept of a Still’s disease continuum that includes both a pediatric/juvenile onset (SJIA) and adult onset (AOSD) form.
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         dateModified:2015-11-20T00:00:00Z
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            Canakinumab
            Gene expression
            Interleukin-1β
            Systemic juvenile idiopathic arthritis
            Pediatrics
            Rheumatology
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ScholarlyArticle:
      headline:Gene-expression analysis of adult-onset Still’s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity
      description:Adult-onset Still’s disease (AOSD), a rare autoinflammatory disorder, resembles systemic juvenile idiopathic arthritis (SJIA). The superimposable systemic clinical features of AOSD and SJIA suggest both clinical phenotypes represent the same disease continuum with different ages of onset. To further characterize the similarity between AOSD and SJIA at the molecular level, 2 previously identified response gene sets in SJIA were used to investigate how genes that respond to interleukin (IL)-1β inhibition with canakinumab in SJIA patients behave in AOSD patients with active disease prior to IL-1β targeting therapy, relative to healthy subjects. All genes downregulated in SJIA patients following canakinumab treatment were upregulated in most patients with active AOSD prior to canakinumab treatment, relative to healthy subjects. A few patients with milder AOSD had expectedly gene-expression patterns that resembled those in healthy subjects. Comparison of the gene-expression patterns with neutrophil counts showed a correlation between elevated neutrophil numbers and upregulation of canakinumab-responsive genes. Correspondingly, most genes upregulated following canakinumab treatment in patients with SJIA patients were downregulated in the majority of AOSD patients. These results further support the concept of a Still’s disease continuum that includes both a pediatric/juvenile onset (SJIA) and adult onset (AOSD) form.
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      dateModified:2015-11-20T00:00:00Z
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         Adult-onset Still’s disease
         Canakinumab
         Gene expression
         Interleukin-1β
         Systemic juvenile idiopathic arthritis
         Pediatrics
         Rheumatology
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            address:
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               type:PostalAddress
            type:Organization
      name:Guido Junge
      affiliation:
            name:Novartis Pharma AG
            address:
               name:Novartis Pharma AG, Basel, Switzerland
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      name:Novartis Institutes for Biomedical Research, Basel, Switzerland
      name:Department of Rheumatology and Clinical Immunology, Charité- Universitätsmedizin, Berlin, Germany
      name:Division of Rheumatology, Department of Medicine, University of Heidelberg, Heidelberg, Germany
      name:Klinik für Rheumatologie & Klinische Immunologie, SJK – University Hospital Essen, Essen, Germany
      name:Division of Rheumatology and Clinical Immunology, Med. Klinik and Poliklinik IV, University of Munich, Munich, Germany
      name:Department of Rheumatology and Clinical Immunology, Charité- Universitätsmedizin, Berlin, Germany
      name:University of Genoa and G Gaslini Institute, Genoa, Italy
      name:Novartis Pharma AG, Basel, Switzerland

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