Journal
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
Volume 11, Issue 5, Pages 575-588Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1586/1744666X.2015.1032257
Keywords
anakinra; biological agents; canakinumab; macrophage activation syndrome; risk factors; systemic juvenile idiopathic arthritis; tocilizumab
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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.
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