What Is Primary Immunodeficiency?

Primary immunodeficiency (PI) is a term used to describe more than 350 disorders. Although they all have differences, what these disorders all have in common is that they cause the immune system to not work right, in some cases making it harder to fight off infections. These infections can happen anywhere in the body, and they can be unusually severe or hard to get rid of.7

In this section you'll learn all about what primary immunodeficiency is, what it is not, and other important facts.

What Primary Immunodeficiency Is Not

Primary immunodeficiency should not be confused with autoimmune disorders or secondary immunodeficiencies. In an autoimmune disorder (like rheumatoid arthritis or Type 1 diabetes), the immune system gets confused and attacks the body.9

With secondary immunodeficiency, something else causes the immune system to not work right, like another infection or a medical treatment (such as chemotherapy). The most common type of secondary immunodeficiency is AIDS, which is caused by the human immunodeficiency virus (HIV).7

primary immunodeficiency should not be confused with autoimmune disorders or secondary immunodeficiencies

With primary immunodeficiency, a part of the immune system is either missing or not working correctly. Primary immunodeficiency diseases are mainly genetic defects that may be inherited.7

PI should not be confused with autoimmune disorders or secondary immunodeficiencies.

How Many People Have Primary Immunodeficiency?

Though they were once thought to be rare, primary immunodeficiencies, as a group, are now considered more common.6 As PI awareness among physicians continues to grow and better diagnostic tools are available, more people are getting diagnosed.6

In a 2007 Immune Deficiency Foundation (IDF) survey, it was estimated that a quarter million people in the United States have been diagnosed with PI, and many more have not yet been diagnosed.10,11 The National Institutes of Health (NIH) estimates that at least half a million people have PI in the United States.12

Quarter million people in the US have been diagnosed with PI Half a million people may have primary immunodeficiency in the US

Primary Immunodeficiency Symptoms and Diagnosis

Susceptibility to infections is one of the most common symptoms of PI.8 However, the symptoms can vary, and they can be mistaken for common infections, since even healthy people without PI can get sick frequently.8

Once thought to be a child's disease, we now know that PI can be diagnosed at any age.7 When testing for PI, doctors will ask about your family history and do a physical exam. They will also order blood tests to check if your blood cells, antibodies, and immune system are working properly. These tests are important for your doctor to confirm a diagnosis of PI and to determine the type of PI you have.7

Download these 10 Warning Signs of PI from the Jeffrey Modell Foundation.

Treating Primary Immunodeficiency

If left untreated, PI can lead to recurring infections.7 However, there are several medical treatments available to help people with PI manage their condition. Talk to your doctor to choose the treatment that is best for you.

Please expand for Indication and Important Safety Information.

IMPORTANT SAFETY INFORMATION

What is the most important information that I should know about HYQVIA?

  • HYQVIA can cause blood clots.
  • Call your healthcare professional (HCP) if you have pain, swelling, warmth, redness, or a lump in your legs or arms, other than at the infusion site(s), unexplained shortness of breath, chest pain or discomfort that worsens on deep breathing, unexplained rapid pulse, numbness or weakness on one side of the body.

HYQVIA is a liquid medicine that is given under the skin (subcutaneously) to treat primary immunodeficiency (PI) in adults.

Important Safety Information
    • HYQVIA can cause blood clots.
    • Call your healthcare professional (HCP) if you have pain, swelling, warmth, redness, or a lump in your legs or arms, other than at the infusion site(s), unexplained shortness of breath, chest pain or discomfort that worsens on deep breathing, unexplained rapid pulse, numbness or weakness on one side of the body.
    • Your HCP may perform blood tests regularly to check your IgG level.
    • With your consent, your HCP may provide blood samples to Shire plc, to test for antibodies that may form against the hyaluronidase part of HYQVIA.
    • Do not infuse HYQVIA into or around an infected or red swollen area because it can cause infection to spread.
    • Talk to your HCP if you become pregnant. Women who become pregnant during HYQVIA treatment are encouraged to enroll in the HYQVIA Pregnancy Registry by calling 1-866-424-6724.

    Do not take HYQVIA if you:

    • Are allergic to IgG, hyaluronidase, other blood products, or any ingredient in HYQVIA.
    • HYQVIA can make vaccines (like measles/mumps/rubella or chickenpox vaccines) not work as well for you. Before you get any vaccines, tell your HCP that you take HYQVIA.

    Before starting HYQVIA, tell your HCP if you:

    • Have or had any kidney, liver, or heart problems or history of blood clots because HYQVIA can make these problems worse.
    • Have IgA deficiency or a history of severe allergic reactions to IgG or other blood products.
    • Are pregnant, trying to become pregnant or are breast feeding.

    HYQVIA can cause serious side effects. If any of the following problems occur after starting HYQVIA, stop the infusion immediately and contact your HCP or call emergency services:

    • Hives, swelling in the mouth or throat, itching, trouble breathing, wheezing, fainting or dizziness. These could be signs of a serious allergic reaction.
    • Bad headache with nausea, vomiting, stiff neck, fever, and sensitivity to light. These could be signs of irritation and swelling of the lining around your brain.
    • Reduced urination, sudden weight gain, or swelling in your legs. These could be signs of a kidney problem.
    • Pain, swelling, warmth, redness, or a lump in your legs or arms, other than at the infusion site(s). These could be signs of a blood clot.
    • Brown or red urine, fast heart rate, yellow skin or eyes. These could be signs of a liver or blood problem.
    • Chest pain or trouble breathing, blue lips or extremities. These could be signs of a serious heart or lung problem.
    • Fever over 100°F. This could be a sign of an infection.

    After HYQVIA infusion a temporary, soft swelling may occur around the infusion site, which may last 1 to 3 days, due to the volume of fluid infused. The following possible side effects may occur at the site of infusion and generally go away within a few hours, and are less likely after the first few infusions.

    • Mild or moderate pain
    • Redness
    • Swelling
    • Itching

    The most common side effects of HYQVIA are:

    • Headache
    • Fatigue
    • Nausea
    • Fever
    • Vomiting

    These are not all the possible side effects. Talk to your HCP about any side effect that bothers you or that does not go away.

    For additional safety information, click for Information For Patients and discuss with your HCP.

    You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.