The definition mainly relies on similarities with monogenic, hereditary periodic fever syndromes, which were at the origin of the concept. By contrast, no specific biomarker exists for systemic autoinflammatory disorders. The diagnosis of autoimmune diseases is often supported by the presence of autoantibodies or autoantigen-specific T cells and B cells. These categories represent a continuum, which enabled a new classification of immune-mediated inflammatory disorders to be refined in subsequent years 3, 5. The term autoinflammation was used to refer to innate immunity and was defined as dysregulated activation of macrophages and neutrophils in response to a danger signal leading to tissue damage. The term autoimmunity was used to refer to adaptive immunity and was defined as aberrant dendritic cell, B cell and T cell responses in primary and secondary lymphoid organs leading to a break in tolerance and development of immune reactivity towards native antigens (with autoantibodies in most cases). In the mid-2000s the hypotheses of two main pathogenic mechanisms underlying immune-mediated inflammation against the self were formulated, and a new classification for immunological diseases was proposed that distinguished autoimmunity from autoinflammation 3, 6. Two IL-1 antagonists have been approved for treatment of AoSD, and growing evidence suggests that other biologic agents are therapeutic options, such as anti-IL-6 and anti-IL-18 therapeutics.Īs a reliable prediction of response and outcome is not possible, therapeutic decisions have to be made on the basis of clinical, biological or imaging characteristics of disease.Ī close cooperation between paediatric and adult rheumatologists is required to establish common diagnostic strategies, treatment targets and goals. Similar to systemic-onset juvenile idiopathic arthritis, adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease with potentially severe inflammatory onset accompanied by a broad spectrum of disease manifestation and complications.ĪoSD should be considered in patients with persistent fever, and the diagnosis is based on the combination of clinical and laboratory findings as well as the exclusion of other inflammatory conditions.Ĭentral to the pathogenesis of AoSD is the intense activation of innate immune cells and overproduction of several pro-inflammatory cytokines including IL-1, IL-6 and IL-18.
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