Hyperimmunoglobinemia D with recurrent fever
Overview
Hyperimmunoglobulinemia D with recurrent fever (commonly abbreviated as HIDS) is a periodic fever syndrome originally described in 1984 by the internist Prof. Jos van der Meer, then at Leiden University Medical Centre. No more than 300 cases have been described worldwide.
Symptoms
* High blood immunoglobulin D level * Recurring periods of fever * Chills * Headache * Swollen glands
Causes
Virtually all patients with the syndrome have mutations in the gene for mevalonate kinase, which is part of the HMG-CoA reductase pathway, an important cellular metabolic pathway (Drenth et al 1999, Houten et al 1999). Indeed, similar fever attacks (but normal IgD) have been described in patients with mevalonic aciduria - an inborn error of metabolism now seen as a severe form of HIDS.
Diagnosis
Home medical testing related to Hyperimmunoglobinemia D with recurrent fever: * Cold & Flu: Home Testing: o Home Fever Tests o Home Ear Infection Tests o Home Flu Tests
Treatment
The recurring fevers are highly unpleasant for patients, but so far only the immunosuppressant drugs etanercept (Enbrel) and anakinra have been shown to be effective. Statin drugs might decrease the level of mevalonate and are presently being investigated.