Hyper-Igd Syndrome

About Hyper IgD Syndrome (HIDS), one of the Mevalonate Kinase Deficiency (MKD) illnesses. Hyper-IgD syndrome (HIDS), or HIDS disease, is caused by an inherited autosomal recessive gene mutation of the mevalonate kinase gene (MVK). Most patients have two mutations on the MVK gene, one from each parent to cause HIDS disease symptoms. However, there are some patients with only one identified mutation on the MVK gene, and some cases where no MVK…

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PFAPA vs. HIDS

Updated 2/9/2018 PFAPA and HIDS have more in common than just a recurring fever. HIDS (hyper-IgD syndrome, a mevalonate kinase deficiency) and PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) are both periodic fever syndromes that generally start in childhood and have very similar symptoms. It can be difficult to distinguish PFAPA from HIDS clinically. This is because the fever length, frequency, and main symptoms…

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High IgD in Periodic Fever Syndromes

“Recent research shows that IgD is an important immunomodulator that orchestrates an ancestral surveillance system at the interface between immunity and inflammation.” 1 Photo by designua/Bigstockphoto.com In 1984, Dr. Jos van der Meer published a study describing a newly recognized periodic fever syndrome. All six patients in this study had similar symptoms of periodic fevers with other inflammatory symptoms. They also all had…

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