2018
DOI: 10.1038/s41584-018-0081-x
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Mechanisms, biomarkers and targets for adult-onset Still’s disease

Abstract: Adult-onset Still's disease (AoSD) is a rare but clinically well-known, polygenic, systemic autoinflammatory disease. Owing to its sporadic appearance in all adult age groups with potentially severe inflammatory onset accompanied by a broad spectrum of disease manifestation and complications, AoSD is an unsolved challenge for clinicians with limited therapeutic options. This Review provides a comprehensive insight into the complex and heterogeneous nature of AoSD, describing biomarkers of the disease and its p… Show more

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Cited by 291 publications
(344 citation statements)
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“…The most consistent diagnosis for our patient was MAS, as he had high persistent fevers without an infectious cause, lymphopenia and anemia, marked hyperferritinemia, hypertriglyceridemia, splenomegaly, and the presence of hemophagocytes on bone marrow biopsy. The diagnosis of The image on the right shows a histiocyte with an engulfed neutrophil adult-onset Still's disease (AoSD) was excluded due to his axSpA [6]. The leading explanations for his MAS were adalimumab, an undetected viral infection, or his underlying axSpA.…”
Section: Discussionmentioning
confidence: 99%
“…The most consistent diagnosis for our patient was MAS, as he had high persistent fevers without an infectious cause, lymphopenia and anemia, marked hyperferritinemia, hypertriglyceridemia, splenomegaly, and the presence of hemophagocytes on bone marrow biopsy. The diagnosis of The image on the right shows a histiocyte with an engulfed neutrophil adult-onset Still's disease (AoSD) was excluded due to his axSpA [6]. The leading explanations for his MAS were adalimumab, an undetected viral infection, or his underlying axSpA.…”
Section: Discussionmentioning
confidence: 99%
“…ASD is a systemic autoin ammatory disease characterized by spiking fever, arthralgia and skin rash, similar to systemic-onset juvenile idiopathic arthritis (sJIA) [4]. This disease is characterized by a dysregulated cytokine network [19].…”
Section: Discussionmentioning
confidence: 99%
“…ASD is considered to be an autoin ammatory disease because of the absence of autoantibodies, similar to other autoin ammatory diseases [2]. Although numerous studies have described potential biomarkers, none have been validated in clinical research, except the IL-1 family cytokine IL-18 [3,4]. IL-18 was originally described as an IFN-g-inducing factor primarily produced by activated macrophages [5].…”
Section: Introductionmentioning
confidence: 99%
“…Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disorder (Feist, Mitrovic, & Fautrel, 2018) of mostly unknown etiology.…”
Section: Introductionmentioning
confidence: 99%
“…It is thought to belong to the spectrum of multigenic autoinflammatory diseases (Feist et al, 2018).…”
Section: Introductionmentioning
confidence: 99%