Welcome to this website

Content last updated on: April 21, 2013


Voor informatie in het Nederlands: zie www.mijnzorgnet.nl/keer_op_keer_koorts - met o.a. een pagina met informatie over HIDS.





This information is meant primarily for people with the hyper-IgD syndrome (also abbreviated to HIDS). Here you'll find among other things general information on the disease, the tests that can be done, what is known about treatment and about heredity. You may come to some information which does not directly concerns you, but which applies to others with this disease. It might be useful for your partner, relatives and friends to read this brochure as well. That will make it easier to think about and discuss the disease.


HIDS is a rare disorder. Many doctors will therefore hardly know about this syndrome, if at all. At the department of General Internal Medicine of the University Medical Center St. Radboud, Nijmegen, in the Netherlands, we have expertise and interest in HIDS and other periodic fever syndromes.


Physician-scientists at our department with clinical expertise in HIDS at the moment are dr. Anna Simon, dr. Marcel van Deuren, dr. Frank van de Veerdonk, dr. Chantal Bleeker and prof.dr. Mihai Netea. Prof.dr. Jos W.M. van der Meer, who first described HIDS in 1984 and has worked on it for years has officially retired in April 2012, but is still actively involved in research.


If you have questions after reading this information, don't hesitate to get answers. You can contact us or of course your own specialist or family doctor. We would also like to hear any comments on or suggestions for improvement for this website. Our e-mail address is email_at_hids.net.


HIDS in brief

The hyper-IgD syndrome, which can yet be abbreviated to HIDS, is a rare disorder involving repeated episodes of high fever accompanied by other signs of inflammation, like swollen lymph nodes, fatigue, headache, muscle ache and stomach ache. Such a fever attack usually lasts a few days, after which it disappears spontaneously. In blood samples, high levels of a certain protein, the IgD, are found. It is a hereditary disease, and DNA-tests can be performed to diagnose the syndrome.


Since mutations are located in the gene for mevalonate kinase (and since not 100% of patients have a high IgD), in recent years the name "mevalonate kinase deficiency" (or MKD) is more and more used. However, it has to be realized that the syndrome MKD encompasses more different symptom complexes than that seen in the original description of HIDS.