Hyper Ig-D syndrome (HIDS)
OMIM number: 260920
Comments closing date: 10/04/2026
Mevalonate kinase deficiency (MKD) is a rare genetic condition that causes the body to have episodes of inflammation without a clear reason. It happens because part of the immune system—the part that reacts quickly to infections—doesn’t work properly. This is different from autoimmune diseases, where the body attacks its own healthy tissues. MKD can be mild or severe. The milder form is called Hyper IgD Syndrome (HIDS). People with HIDS have repeated attacks of high fever, which can last a few days. These episodes often come with symptoms like joint and muscle pain, skin rashes, and tummy pain. The number and severity of attacks can be very different from person to person. The more severe form is called mevalonate aciduria. In addition to fever attacks, it can cause poor growth, developmental problems, and eye issues. Children with this form can sometimes experience serious, life-threatening complications. MKD is caused by changes in a gene called MVK, which affects important chemical processes in the body.
