Question 1

What is Hyper IgD syndrome (HIDS)?

Accepted Answer

Hyper IgD syndrome (HIDS) is a rare inherited disorder that causes recurrent episodes of fever, abdominal pain, joint pain, and inflammatory symptoms. It is caused by genetic mutations in the MVK gene that disrupt immune system regulation and mevalonate kinase enzyme production. The IgD blood test is the most important test for diagnosis because it detects the characteristically elevated immunoglobulin D levels in HIDS patients.

Question 2

What causes Hyper IgD syndrome (HIDS)?

Accepted Answer

Hyper IgD syndrome is caused by mutations in the MVK gene, which provides instructions for making the mevalonate kinase enzyme. These genetic mutations are inherited in an autosomal recessive pattern, meaning both parents must carry the defective gene for a child to develop HIDS. The defective enzyme disrupts cholesterol production pathways and triggers abnormal immune responses, leading to periodic inflammatory episodes and chronically elevated immunoglobulin D levels in the bloodstream.

Question 3

What is the best test for Hyper IgD syndrome?

Accepted Answer

The IgD blood test is the most important test for Hyper IgD syndrome because it detects elevated immunoglobulin D levels that are characteristic of this condition. Most HIDS patients show IgD levels above 100 IU/mL, compared to normal levels below 10 IU/mL. This test is typically combined with genetic testing for MVK gene mutations and measurement of IgA levels, which are also often elevated in HIDS. During fever episodes, inflammatory markers like C-reactive protein and white blood cell counts may also be elevated, but the IgD test remains the key diagnostic marker for confirming this rare genetic disorder.

Question 4

When should I get tested for Hyper IgD syndrome?

Accepted Answer

You should get tested if you or your child experiences recurring fever episodes that last 3-7 days and occur every 4-8 weeks, especially when accompanied by abdominal pain, joint pain, skin rashes, or swollen lymph nodes. Testing is particularly important if fever episodes began in infancy or early childhood, if there is a family history of similar symptoms, or if standard treatments for common infections have not been effective. Early diagnosis helps distinguish HIDS from other periodic fever syndromes and guides appropriate treatment to reduce inflammation and improve quality of life.

Question 5

What are the symptoms of Hyper IgD syndrome?

Accepted Answer

Hyper IgD syndrome causes recurrent fever episodes that typically last 3-7 days and occur every 2-8 weeks, often starting before age one. During attacks, you may experience high fever above 104°F, severe abdominal pain with vomiting and diarrhea, painful swollen joints, skin rashes or lesions, swollen lymph nodes in the neck, headaches, and general fatigue. Some people also develop mouth sores, muscle pain, and enlarged liver or spleen. Between fever episodes, most people feel completely normal with no symptoms, though the unpredictable nature of attacks can significantly impact daily life and activities.

Question 6

Who is at risk for Hyper IgD syndrome?

Accepted Answer

Hyper IgD syndrome primarily affects people of Dutch and French ancestry, though it can occur in any ethnic group. The condition is inherited in an autosomal recessive pattern, meaning both parents must carry the MVK gene mutation for their child to develop HIDS. Children with two carrier parents have a 25% chance of inheriting the condition. HIDS typically appears in infancy or early childhood, with most cases diagnosed before age one. Family history of periodic fever syndromes or unexplained recurrent fevers increases risk, and genetic counseling is recommended for families with known carriers or affected members.

Question 7

What happens if Hyper IgD syndrome is left untreated?

Accepted Answer

Untreated Hyper IgD syndrome can lead to chronic inflammation that affects multiple organ systems and significantly impacts quality of life. Repeated inflammatory episodes may cause joint damage and arthritis, developmental delays in children due to frequent illness, gastrointestinal complications including inflammatory bowel symptoms, and increased risk of amyloidosis where abnormal protein deposits damage kidneys and other organs. The unpredictable fever attacks can interfere with school, work, and social activities, leading to psychological stress and social isolation. While HIDS is not typically life-threatening, proper diagnosis and treatment are essential to reduce inflammation, prevent complications, and improve daily functioning.

Question 8

Can Hyper IgD syndrome be diagnosed with a blood test?

Accepted Answer

Yes, Hyper IgD syndrome can be diagnosed through blood testing combined with genetic analysis. The IgD blood test measures immunoglobulin D levels, which are typically elevated above 100 IU/mL in HIDS patients compared to normal levels below 10 IU/mL. Blood tests also check IgA levels, which are often elevated, and inflammatory markers like C-reactive protein and erythrocyte sedimentation rate during fever episodes. Genetic testing that identifies MVK gene mutations confirms the diagnosis. Your doctor may order blood tests both during fever attacks and between episodes to see the full picture, as some markers fluctuate while IgD levels remain consistently elevated.

Question 9

How is Hyper IgD syndrome treated?

Accepted Answer

Hyper IgD syndrome is treated with medications that reduce inflammation and control immune system overactivity. Biologic drugs like canakinumab and anakinra that block interleukin-1, a key inflammatory protein, have shown the most success in reducing fever frequency and severity. During acute attacks, nonsteroidal anti-inflammatory drugs and corticosteroids can help manage symptoms. Some patients benefit from colchicine or statins for ongoing inflammation control. Treatment is highly individualized and typically managed by rheumatologists or immunologists experienced with autoinflammatory disorders. While there is no cure, proper treatment can significantly reduce attack frequency and severity, allowing most people to lead relatively normal lives.

Question 10

How can I prevent Hyper IgD syndrome?

Accepted Answer

Hyper IgD syndrome cannot be prevented because it is an inherited genetic condition caused by mutations in the MVK gene. However, genetic counseling can help families understand their risk before having children. If both parents are known carriers of MVK gene mutations, prenatal genetic testing and preimplantation genetic diagnosis during in vitro fertilization are options to identify affected embryos. For people already diagnosed with HIDS, preventing fever attacks involves avoiding known triggers when possible, such as vaccinations, stress, minor infections, or physical trauma, though triggers vary by individual and attacks often occur without clear causes. Focus on early diagnosis and proper treatment rather than prevention.

Question 11

What can I do at home for Hyper IgD syndrome?

Accepted Answer

At home, you can manage HIDS symptoms by staying well-hydrated during fever episodes, using cool compresses to reduce fever discomfort, and getting plenty of rest when attacks occur. Keep a detailed symptom diary tracking fever patterns, triggers, and symptom severity to help your doctor optimize treatment. Apply heat or cold packs to painful joints and maintain a balanced diet rich in anti-inflammatory foods like fish, fruits, and vegetables. Stress management techniques including meditation, gentle exercise between attacks, and adequate sleep may help reduce attack frequency. Stay current with prescribed medications and maintain regular follow-up with your specialist, as consistent medical management is essential for controlling this genetic condition.

Question 12

How’s this work?

Accepted Answer

Getting your blood test with us is easy, private & backed by the power of science.Long story short:
            
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                When you’re ready, visit one of our 4,000 locations. Just bring your ID – no printing or faxing of your lab order is needed, as your order and details will already be in the lab system.
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Question 13

How do I know which test to get?

Accepted Answer

In the test options, find the test you want.
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            Just text or call us at 754-799-7833, email us at support@privatemdlabs.com, and we'll gladly help you. We've got your back and reply quickly.

Question 14

When will my lab results be available?

Accepted Answer

Most test results will be available within 1–3 business days. Once they're ready, we'll send an email and text message to let you know.
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Question 15

Is it possible to make changes to my lab order if I made a mistake with the name, date of birth, or any other details?

Accepted Answer

Absolutely! We totally understand that errors can happen. No worries, we're here to help you.
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            Our lab requires an address to be listed to generate an order.

Question 16

Do you accept health insurance?

Accepted Answer

Only HSA & FSA is accepted.
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Question 17

Can I cancel my order?

Accepted Answer

Yes.
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Question 18

How can I find a lab location near me?

Accepted Answer

During the ordering process, you’ll be able to select a specific lab near you, with no strings attached!
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Hyper IgD Syndrome (HIDS) Blood Test

What is Hyper IgD syndrome (HIDS)?

What causes Hyper IgD syndrome (HIDS)?

What is the best test for Hyper IgD syndrome?

When should I get tested for Hyper IgD syndrome?

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