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• W4312687580 endingPage "1429" @default.
• W4312687580 startingPage "1399" @default.
• W4312687580 abstract "The systemic autoinflammatory diseases comprise a clinically heterogenous group of rare conditions, typically characterized by episodes of systemic and localized inflammation. Emerging understanding of common pathophysiological pathways underlying these conditions has led to the design and application of targeted therapies in many of these diseases. The inherited forms present in childhood or early adult life with episodic inflammatory symptoms which most often manifest as attacks of fever accompanied by at least some of myalgia and arthralgia, rash, aphthous ulcers, lymphadenopathy, abdominal or chest pain which can be serositis, transient florid arthritis. Family history is seen in more than 6% of autosomal dominant diseases but may be absent in the recessive types. Acquired forms are present later in life with no family history. For general physicians, whilst unfamiliarity with these rare diseases and their therapies may be daunting, an awareness of these conditions is important, as these are high-value diagnoses due to the potential for effective treatment. Such patients may be encountered in a variety of settings, from general practice to the acute surgical take to the perinatal clinic. As such, appreciation of the principles of management of these patients’ systemic autoinflammatory disease is important for the general physician to treat any acute presenting problem, whilst also optimizing the long-term management of their underlying condition and preventing potentially devasting complications such as visual loss or renal failure due to AA amyloidosis. Here we provide a practical resource for clinicians of any specialty in the care of patients with these conditions by addressing: the principles of management of the four best characterized systemic autoinflammatory diseases, the therapeutic agents in use, and the nonpharmacological aspects of management." @default.
• W4312687580 created "2023-01-05" @default.
• W4312687580 creator A5001460025 @default.
• W4312687580 creator A5020344924 @default.
• W4312687580 date "2022-01-01" @default.
• W4312687580 modified "2023-10-18" @default.
• W4312687580 title "Management of autoinflammatory syndromes and periodic fevers" @default.
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