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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Bruck, Normi Schnabel, Anja Hedrich, Christian M. |
| Description | Country affiliation: Germany Author Affiliation: Bruck N ( Pediatric Rheumatology and Immunology, Children's Hospital Dresden, Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Germany.); Schnabel A ( Pediatric Rheumatology and Immunology, Children's Hospital Dresden, Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Germany.); Hedrich CM ( Pediatric Rheumatology and Immunology, Children's Hospital Dresden, Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Germany. Electronic address: Christian.hedrich@uniklinikum-dresden.de.) |
| Abstract | Because of its distinct clinical and laboratory features, reflecting systemic inflammation, sJIA can be distinguished from other forms of JIA which usually present as a milder phenotype. The exact pathophysiology of sJIA, however, remains unknown. Profound dysregulation of innate pro- and anti-inflammatory cytokines, and rapid clinical response to cytokine blocking strategies in sJIA patients suggest impaired control mechanisms in innate immune cells contributing to sJIA pathogenesis. Endogenous TLR ligands, such as S100 protein complexes, enhance the pro-inflammatory phenotype. Associations with polymorphisms in cytokine genes and their receptors suggest a genetic component. Furthermore, genetic associations that have been reported in familial hemophagocytic lympohistiocytosis also exist in patients with sJIA-associated macrophage activation syndrome, a severe complication of sJIA. Reported mutations in single genes, however, are too weak to confer sJIA, suggesting a multi-factorial mode of inheritance. We provide an overview of current pathophysiological concepts, state-of-the-art treatment regimens, and unanswered questions in sJIA. |
| File Format | HTM / HTML |
| ISSN | 15216616 |
| Issue Number | 1 |
| Volume Number | 159 |
| e-ISSN | 15217035 |
| Journal | Clinical Immunology |
| Language | English |
| Publisher | Elsevier |
| Publisher Date | 2015-07-01 |
| Publisher Place | United States |
| Access Restriction | One Nation One Subscription (ONOS) |
| Subject Keyword | Discipline Immunology Arthritis, Juvenile Immunology Cytokines Macrophage Activation Syndrome Antibodies, Monoclonal Therapeutic Use Antibodies, Monoclonal, Humanized Antirheumatic Agents Drug Therapy Genetics Glucocorticoids Humans Interleukin 1 Receptor Antagonist Protein Recombinant Fusion Proteins Tumor Necrosis Factor-alpha Antagonists & Inhibitors Journal Article Review |
| Content Type | Text |
| Resource Type | Article |
| Subject | Immunology and Allergy Immunology |
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