title: Complications of systemic juvenile idiopathic arthritis: risk factors and management recommendations.
abstract: Systemic juvenile idiopathic arthritis (SJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. Systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that SJIA is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of SJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective. However, complications of SJIA, including macrophage activation syndrome, limitations in functional outcome by arthritis and long-term damage from chronic inflammation, continue to be a major issue in SJIA patients' care. Translational research leading to a profound understanding of the cytokine crosstalk in SJIA and the identification of risk factors for SJIA complications will help to improve long-term outcome.
authors.affiliation: Pediatric Rheumatology, University of Basel, University Children's Hospital, Basel, Switzerland.
authors.forename: Andreas
authors.initials: A
authors.lastname: Woerner
authors.forename: Annette
authors.initials: A
authors.lastname: von Scheven-Gête
authors.forename: Rolando
authors.initials: R
authors.lastname: Cimaz
authors.forename: Michaël
authors.initials: M
authors.lastname: Hofer
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D001171:Arthritis, Juvenile / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007113:Immunity, Innate; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D007375:Interleukin-1 / Q000276:immunology*; D015850:Interleukin-6 / Q000276:immunology*; D008262:Macrophage Activation; D012307:Risk Factors; D057170:Translational Research, Biomedical
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D053590:Interleukin 1 Receptor Antagonist Protein; D007375:Interleukin-1; D015850:Interleukin-6; C541220:canakinumab; C502936:tocilizumab
keywords: anakinra; biological agents; canakinumab; macrophage activation syndrome; risk factors; systemic juvenile idiopathic arthritis; tocilizumab