publication . Article . 2014

Adult-onset Still's disease

Mathieu Gerfaud-Valentin; Yvan Jamilloux; Jean Iwaz; Pascal Sève;
Open Access English
  • Published: 01 Jul 2014
  • Publisher: HAL CCSD
  • Country: France
Abstract
International audience; First described in 1971, adult-onset Still's disease (AOSD) is a rare multisystemic disorder considered as a complex (multigenic) autoinflammatory syndrome. A genetic background would confer susceptibility to the development of autoinflammatory reactions to environmental triggers. Macrophage and neutrophil activation is a hallmark of AOSD which can lead to a reactive hemophagocytic lymphohistiocytosis. As in the latter disease, the cytotoxic function of natural killer cells is decreased in patients with active AOSD. IL-18 and IL-1β, two proinflammatory cytokines processed through the inflammasome machinery, are key factors in the pathogen...
Subjects
free text keywords: Macrophage activation syndrome, Innate, Immunity, Adaptive Immunity, Adult, Adult-onset Still's disease, Anakinra, Autoinflammatory disorder, Genetic Predisposition to Disease, Hemophagocytic lymphohistiocytosis, Humans, Hyperferritinemia, Prognosis, Still's Disease, Adult-Onset, [SDV.IMM]Life Sciences [q-bio]/Immunology, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, Immunology, Immunology and Allergy, lcsh:Diseases of the musculoskeletal system, lcsh:RC925-935, Arthritis, medicine.disease, medicine, Macrophage activation syndrome, Tocilizumab, chemistry.chemical_compound, chemistry, Hemophagocytic lymphohistiocytosis, Autoinflammatory Syndrome, Immunology, business.industry, business, Proinflammatory cytokine, Disease, Anakinra, medicine.drug
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