Gene-expression analysis of adult-onset Still’s disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity
| Autor: | Jan Zernicke, Eugen Feist, Guido Junge, Arndt Brachat, Christof Specker, M. Witt, N. R. Nirmala, Alberto Martini, Norbert Blank |
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| Rok vydání: | 2015 |
| Předmět: |
Adult-Onset
Male Interleukin-1beta Medizin Juvenile Gene Expression Arthritis Disease 600 Technik Medizin angewandte Wissenschaften::610 Medizin und Gesundheit 610 Medical sciences Medicine Systemic juvenile idiopathic arthritis Monoclonal Immunology and Allergy Child Adult-onset Still’s disease Antibodies Monoclonal Interleukin Interleukin-1β Female Still's Disease Adult-Onset medicine.drug Adult medicine.medical_specialty Canakinumab Short Report Adult-onset Still's disease Antibodies Monoclonal Humanized Antibodies Rheumatology Internal medicine medicine Humans Pediatrics Perinatology and Child Health business.industry Gene Expression Profiling Case-control study medicine.disease Still's Disease Arthritis Juvenile Gene expression profiling Case-Control Studies Gene expression Pediatrics Perinatology and Child Health Immunology business |
| Zdroj: | Pediatric Rheumatology Online Journal |
| ISSN: | 1546-0096 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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