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Hyper IgD Syndrome (HIDS)
Wednesday, 16 July 2003
Monday, 29 November 2004
Periodic Fever Syndrome


Hyper IgD is a rare inherited disorder that primarily causes recurrent attacks of fever, along with joint inflammation, tiredness, headache, muscle aches, and stomachache.


This disorder begins in children most often during infancy, usually before 1 year of age, although the symptoms may also appear for the first time later in life. It affects both females and males equally, and the number of people with this disorder worldwide is around 200. Most of the people with this disorder are of Dutch or French ancestry. The disease is autosomal recessive, meaning that the child must have one defective gene from each parent.

Signs and Symptoms

Recurring “attacks” of fever, up to 104 degrees, ranging from several times a month to every 4-8 weeks, with a 3-7 day duration. Swollen glands, commonly found in neck. Sore throat, or small ulcers in the mouth. Rash Pain in muscles and joints. Headache, stomachache. Nausea, vomiting, and diarrhea. Chills preceding fever. Loss of appetite.

Possible Causes

The exact cause of this disorder has not been found, but it is known that there is a broken protein, called mevalonate kinase, that does not work properly in these children. Research has not determined how this protein is involved with inflammation and fever, although it is thought to possibly play a role in normal inflammatory reactions, where the body tries to get rid of substances that do not belong in the body.


This is a rare diagnosis suspected when a child has recurring episodes of fever with an unknown cause, which last a few days and then suddenly stops. The child may also have a brother or sister with the same symptoms. There are a couple of ways that your doctor can diagnose your child with Hyper IgD syndrome. One way is to take a sample of your child’s blood, and look for high levels of a protein called IgD. Although everyone who has hyper-IgD syndrome has high IgD levels, not everyone with high IgD levels has hyper-IgD syndrome. There are many other causes. Therefore, all the components of the syndrome have to be present to make a diagnosis. Since Hyper IgD is an inherited disorder that has been located in our genes, doctors can also look for it by doing a DNA analysis test. Patients with Hyper IgD will have a unique change in their DNA, and doctors can find this change and diagnose your child. This test takes longer than a month, so you have to be patient while the laboratory finishes the test.


Children with Hyper IgD currently use non-specific drugs such as acetaminophen to help with the pain and fever symptoms. Sometimes low dose steroids, like prednisone, are used and are helpful, but have side effects. Unfortunately, there is no other effective treatment at this time. Although no cure has been found, research is being performed in an attempt to find the exact cause and appropriate treatment for these individuals.


Patients with Hyper IgD live a normal life span, although they experience recurring episodes of fever throughout their life. However, most children with Hyper IgD do not feel ill when they do not have a fever episode. Also, this disorder does not directly affect the development or growth of your child.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.


Medicine Net
A very brief but nicely written summary of the syndrome.

Google Search for Hyper IgD Syndrome (HIDS)

References and Sources