Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 144, Issue 3, Pages 279-302Publisher
WILEY
DOI: 10.1111/j.1365-2141.2008.07036.x
Keywords
periodic fever; autoinflammatory syndrome; innate immunity; interleukin-1; inflammasome
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Funding
- Netherlands Organization for Health Research and Development [912-03-024]
- ZonMw VENI
- [016.066.082]
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The hereditary periodic fever syndromes encompass a rare group of diseases that have lifelong recurrent episodes of inflammatory symptoms and an acute phase response in common. Clinical presentation can mimic that of lymphoproliferative disorders and patients often go undiagnosed for many years. These syndromes follow an autosomal inheritance pattern, and the major syndromes are linked to specific genes, most of which are involved in regulation of the innate immune response through pathways of apoptosis, nuclear factor kappa Beta activation and cytokine production. In others, the link between the protein involved and inflammation is less clear. The recurrent inflammation can lead to complications, such as renal impairment due to amyloidosis and vasculitis, visual impairment, hearing loss, and joint destruction, depending on the specific syndrome. In recent years, treatment options for these diseases have improved significantly. Early establishment of an accurate diagnosis and start of appropriate therapy improves prognosis in these patients.
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