What Is Primary Immunodeficiency?

Primary immunodeficiency (PI) is a term used to describe more than 300 disorders.6 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 and skin 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.

Selected Important Safety Information about HYQVIA
  • HYQVIA can cause blood clots. Call your healthcare professional 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.
  • Do not take HYQVIA if you are allergic to IgG, hyaluronidase, other blood products, human albumin (in the hyaluronidase solution), or have IgA deficiency with antibodies to IgA.
Indication and Important Safety Information
HYQVIA [Immune Globulin Infusion 10% (Human) with Recombinant Human Hyaluronidase] Solution
  • HYQVIA is a liquid medicine containing immune globulin and Recombinant Human Hyaluronidase. HYQVIA contains IgG antibodies, collected from human plasma donated by healthy people. The antibodies help your body to fight off bacterial and viral infections. The hyaluronidase part of HYQVIA helps more of the immune globulin get absorbed into the body to fight infection.
  • HYQVIA is indicated for the treatment of Primary Immunodeficiency (PI) involving the humoral immune system in adults.
  • Safety and efficacy of chronic use of Recombinant Human Hyaluronidase in HYQVIA have not been established in conditions other than PI.
  • HYQVIA is infused under the skin (subcutaneous).
  • If you and your healthcare professional decide that home self-infusion of HYQVIA is right for you, then be sure you get instructions and training from your healthcare professional before using HYQVIA at home.
Important Safety Information
    • HYQVIA can cause blood clots.
    • Call your healthcare professional 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 healthcare professional may perform blood tests regularly to check your IgG level.
    • With your consent, your healthcare professional 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 healthcare professional if you become pregnant. Women who become pregnant during HYQVIA treatment are encouraged to enroll in the HYQVIA Pregnancy Registry by calling Medical Information at 1-866-424-6724.

    Do not take HYQVIA if you:

    • Are allergic to IgG, hyaluronidase, other blood products, or human albumin (in the hyaluronidase solution).
    • Have IgA deficiency with antibodies to IgA.

    HYQVIA can cause serious side effects. Call your healthcare professional or go to your emergency department right away if you get:

    • 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 swelling in 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 lung problem.

    What are the possible or reasonably likely side effects of HYQVIA?

    • 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. Mild or moderate pain, redness, swelling, or itching may occur at the site of infusion and generally go away in a few hours. Local reactions are less likely after the first few infusions.
    • The most common side effects of HYQVIA are headache, fatigue, nausea, fever, and vomiting.
    • Antibodies to the hyaluronidase component of HYQVIA were formed in some patients taking HYQVIA. It is not known if there is any long term effect. In theory, these antibodies could react with your body’s own PH20. PH20 is present in the male reproductive tract. So far, these antibodies have not been associated with increased or new side effects.

    These are not all the possible side effects with HYQVIA. Talk to your healthcare professional about any side effects that bother you or that don’t go away.

    Before starting HYQVIA, tell your healthcare professional 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.

    You are encouraged to report suspected side effects by contacting FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or Shire Drug Safety at 1-800-999-1785.

    The risk information provided here is not comprehensive. To learn more, talk about HYQVIA with your healthcare provider or pharmacist.

    Please see the FDA-approved patient labeling