Hyperimmunoglobulinemia D with recurrent fever is a syndrome of fever which was originally described by internist Prof. Jos van der Meer at Leiden University Medical Centre, in 1984. Surveyed worldwide no more than 300 cases all over the world have been traced. Like many other periodic fever syndrome, the HIDS is also one. The symptoms are often marked by patient having fever, arthralgia, skin lesions, and diarrhea. The laboratory features of the disease include an acute phase response. Some cases also have normal IgD have been described.
The syndrome is mainly found in The Netherlands and France. The international registry also includes several cases from other countries. Different diagnosis forms of Hyperimmunoglobulinemia D with recurrent fever include fever of unknown origin, familial Mediterranean fever (FMF) and familial Hibernian fever
Almost all patients with the HIDS syndrome get mutations in the gene for
mevalonate kinase which is also a part of the HMG-CoA reductase pathway. The HMG-CoA reductase is an important cellular metabolic pathway.
Some fever attacks have also been found in the patients with mevalonic aciduria - an inborn error of metabolism. It is now recognized as a severe form of the HIDS.
The frequent fevers of Hyperimmunoglobulinemia D with recurrent fever become difficult for the patients to cope with. But for diagnosing only the immunosuppressant drugs like etanercept and anakinra have been found effective. The statin drugs are also being investigated.
The HIDS with periodic fever syndrome is an inherited and acute fever syndrome. The Mevalonate kinase activity is deficient in children.
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It is also transmitted from a mother infected, to her child. Suppose the mother who was symptomatic during childhood and adolescence. The diseases can not be diagnosed only basing on the inheritance patterns.
People having Hyperimmunoglobulinemia D with recurrent fever face disturbances in leading normal life and have disturbances in the normal flow of their day to day work. It also damages different organs making them partially impaired.
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