Vasculitis in Systemic Autoinflammatory Diseases
نویسندگان
چکیده
منابع مشابه
[Hereditary systemic autoinflammatory diseases].
Systemic autoinflammatory diseases encompass different rare clinical entities characterized by recurrent acute inflammatory episodes secondary to a dysregulated inflammatory process. Since their first clinical descriptions, the Mendelian hereditary nature of some of them became evident, with their genetic and molecular basis being recently elucidated. There are disease-causing mutations in gene...
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The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs), an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1β and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflam...
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Inflammatory diseases of the CNS blood vessels are a large heterogeneous group with multiple causes sharing certain pathological features, particularly intramural inflammation and necrotic changes within the vascular wall. Unlike infectious vasculitides secondary to direct infection of blood vessels by organisms, non-infectious CNS vasculitides appear to result from immunological injury. Noninf...
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Autoinflammatory diseases (AIDs) are characterized by recurrent episodes of systemic and organ-specific inflammation. Many of these diseases share fever as a common presenting feature. Physicians need to consider AIDs in children with recurrent, unexplained fevers, when infectious and malignant causes have been discarded. This article discusses the differential diagnosis of recurrent fever in c...
متن کاملAutoinflammatory diseases in pediatrics.
Monogenic autoinflammatory syndromes are caused by mutations in protein-coding genes that have a pivotal role in the regulation of the inflammatory response. Due to their genetic nature, most of these syndromes usually begin during childhood. They are clinically characterized by recurrent episodes of systemic inflammation (fever with different clinical manifestations, such as skin rash, serosit...
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ژورنال
عنوان ژورنال: Frontiers in Pediatrics
سال: 2018
ISSN: 2296-2360
DOI: 10.3389/fped.2018.00377