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ABOUT PRIMARY IMMUNODEFICIENCY

People with PI have an immune system that does not fight off infections well enough1,2

There are more than 550 conditions of primary immunodeficiency.3

What is PI?

With primary immunodeficiency, part of your immune system, which protects you from infections and fights off diseases, is missing or doesn’t function properly. As a result, your body can’t protect you from germs as well as it should, so you are more likely to develop infections and get sick more often.1,2

 

PI may be inherited, which means it is carried through the genes of your parents. If you have the disease, you might pass it along to your children, but you won’t give it to anyone else.4

 

If you or someone you know has PI, you know how difficult it can be. But there is good news, too. There are 
FDA-approved treatment options available and lots of support. If you have PI, you are certainly not alone.

Journey to Primary Immunodeficiency Diagnosis

Listen as Joanne, a retired registered nurse with PI, describes her journey to getting diagnosed with PI.

Understanding PI symptoms and diagnosis

The signs and symptoms of PI vary from person to person. If someone you know is experiencing any of the symptoms below, encourage them to talk to their doctor. Early detection of PI can help prevent long-term problems.1

If a person is experiencing recurrent and/or chronic infections, lab testing may be necessary to detect the presence of PI. The patient’s medical history and physical exam will help determine the appropriate choice of lab tests. Blood samples and DNA analysis can be used to confirm a particular diagnosis.1

The most common symptoms of PI1,5

  • Chronic, recurring infections, such as sinus infections, bronchitis, and ear infections, which are more severe, longer lasting, and more difficult to cure than is typical 
  • Frequently getting sick with infections, such as a common cold, that a healthy immune system would normally be able to fight off
  • Autoimmune problems, such as autoimmune lung or skin disease, which happen when the immune system attacks the body’s own organs. Other issues include anemia, celiac disease, arthritis, lupus, and thyroid disorders
Complete the PI self-check

What causes PI?

Overview of the immune system

The immune system is an incredible network of cells, proteins, tissues, and organs that defends against a wide variety of germs every day. One important component of the immune system is antibodies.1

How do antibodies work?

Antibody icon

The body’s immune system makes special proteins called antibodies1

Bacteria icon

When the body detects a threat, such as a germ that can make you sick, it responds by making antibodies1

Antibodies attaching to bacteria icon

Antibodies defend the body by attaching to the threat and signaling the body to destroy the germ1

The immune system in people with PI

Usually, the immune system does a great job protecting the body from threats. But in people who have PI, the immune system doesn’t work properly. In some cases, the immune system may not work at all. With a lower amount of antibodies, or antibodies that are not working properly, germs are more likely to get through the body’s defenses.1

IG replacement therapy helps give your immune system infection-fighting antibodies6-8

IG stands for immune globulin. It is made up of proteins taken from human plasma and contains purified antibodies that help your body fight infections.6-8 IG therapy can be given in 2 ways: 

SCIG icon

SCIG

Infused just below the skin (subcutaneously)1

IVIG

Infused through a vein, typically in the arm (intraveneously)1

Starting IG therapy soon after you are diagnosed with PI can help reduce your risk of permanent health issues.8

Not all IG therapies are the same9

IG therapies differ in the way they are made, how they are dosed, and how tolerable they are. 

• It’s important to talk to your doctor about possible side effects before starting IG therapy 

Talk to your doctor about your IG therapy options 

Whether you are new to IG or considering changing your IG treatment, you can make choices about your experience. Here are a few things you can discuss in an open and honest conversation with your doctor: 

If you are new to IG: 

  • Do you want to get your IG infusions at home or in a medical office or infusion center? 
  • Is it more important to you to have shorter infusion times or a longer time between infusions? 

If you are currently taking IG:

  • Do you experience side effects, such as headache or nausea with your treatment? 
  • After your infusions, are you so tired you have to cancel activities that day or even the next day or two? 
Discussion guide

For more tips for talking to your doctor about starting or changing your IG treatment, download this discussion guide. 

Download now

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Terms to Know

DNA, deoxyribonucleic acid; IG, immune globulin; IVIG, intravenous immunoglobulin; PI, primary immunodeficiency disease; SCIG, subcutaneous immunoglobulin.

What is XEMBIFY®?

XEMBIFY® (immune globulin subcutaneous human–klhw) is a 20% immune globulin used in the treatment of primary humoral immunodeficiency disease (PIDD) in patients 2 years of age and older. XEMBIFY is for subcutaneous administration only.

 

IMPORTANT SAFETY INFORMATION

 

WARNING: THROMBOSIS

  • Thrombosis (formation of blood clots within blood vessels) may occur with immune globulin products, including XEMBIFY. Before you take XEMBIFY, talk to your doctor if you:
    • Are older
    • Are sedentary (need to lie down or sit down) for long periods of time
    • Are taking estrogen-containing medicines (birth control pills, hormone replacement therapy)
    • Have a permanent intravenous (IV) catheter
    • Have hyperviscosity of the blood (diseases such as multiple myeloma or other causes of elevated proteins in the blood)
    • Have cardiovascular (heart) problems or previous history of stroke
  • Thrombosis may occur even if you don’t have any risk factors
  • If you are at risk of thrombosis, your doctor may prescribe XEMBIFY at the minimum dose and infusion rate. Make sure you drink plenty of fluid before taking XEMBIFY. Make sure your doctor is checking you regularly for signs and symptoms of thrombosis and is checking your blood viscosity if you are at risk of hyperviscosity

Who should not use XEMBIFY?

  • XEMBIFY should not be used if you have had a severe allergic reaction to human immune globulin, or if you have been told by a doctor that you are IgA deficient and have developed antibodies to IgA and hypersensitivity after exposure to a previous plasma product

What are possible serious side effects of XEMBIFY?

  • Aseptic meningitis syndrome (AMS). Aseptic meningitis is a non-infectious inflammation of the membranes that cover the brain. It causes a severe headache syndrome, which may occur with human immune globulin treatment, including XEMBIFY. If you are showing signs and symptoms of AMS, your doctor may conduct a thorough neurological evaluation including spinal tap (sampling fluid which surrounds the spinal cord) to rule out other causes of meningitis. Stopping human immune globulin treatment has resulted in the end of signs and symptoms within several days. Treatment may include analgesics (pain medicines) and/or a special procedure known as a “blood patch” to stop headache
  • Hypersensitivity. Severe allergic reactions may occur with immune globulin products, including XEMBIFY. If you have a severe allergic reaction, stop the infusion immediately and get medical attention. XEMBIFY contains IgA. If you have known antibodies to IgA, you may have a greater risk of developing potentially severe allergic reactions
  • Kidney problems or failure. Kidney problems or failure may occur with use of human immune globulin products, especially those containing sucrose (sugar). XEMBIFY does not contain sucrose. If you have kidney disease or diabetes with kidney involvement, your doctor should perform a blood test to assess your hydration level and kidney function before beginning immune globulin treatment and at appropriate intervals thereafter. If your doctor determines that kidney function is worsening, they may discontinue treatment
  • Hemolysis. Your doctor should monitor you for symptoms of hemolysis (destruction of red blood cells causing anemia, or low red blood cell count). If your doctor suspects hemolysis, they should perform additional tests to confirm
  • Transfusion-related acute lung injury (TRALI). TRALI is a rare but serious syndrome characterized by sudden acute respiratory distress following transfusion. If your doctor suspects TRALI, they will monitor you for any other lung issues. TRALI may be managed with oxygen therapy
  • Transmissible infectious agents. Because XEMBIFY is made from human blood, it may carry a risk of transmitting infectious agents such as viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. No cases of transmission of viral diseases or CJD have been associated with the use of XEMBIFY
  • Interference with lab tests. Because XEMBIFY contains a variety of antibodies, blood tests to determine antibody levels may be falsely elevated. Be sure to tell your doctor or lab technician that you are using XEMBIFY

What are other possible side effects of XEMBIFY?

  • In clinical studies of XEMBIFY, some patients experienced local side effects (at the injection site) including pain, redness, puffiness, bruising, nodules, itching, firmness, scabbing and swelling at the site on the skin where the injection occurred. Some patients experienced non-injection-site side effects including cough and diarrhea
  • Use of XEMBIFY may interfere with the immune response to virus vaccines, such as vaccines for measles, mumps, rubella and varicella. Tell your doctor you are taking XEMBIFY before getting vaccinations

Please see accompanying full Prescribing Information for XEMBIFY.

 

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.

References

  1. Ballow M, Epland K, Heimall J, et al. Immune Deficiency Foundation Patient & Family Handbook for Primary Immunodeficiency Diseases. 6th ed. Immune Deficiency Foundation; 2019.
  2. Immune Deficiency Foundation. IDF Guide for Nurses: Immunoglobulin Therapy for Primary Immunodeficiency Diseases. 4th ed. Immune Deficiency Foundation; 2016.
  3. Poli MC, Aksentijevich I, Bousfiha AA, et al. Human inborn errors of immunity: 2024 update on the classification from the International Union of Immunological Societies Expert Committee. J Hum Immun. 2025;1(1):e20250003.
  4. Meyts I, Bousfiha A, Duff C, et al. Primary immunodeficiencies: a decade of progress and a promising future. Front Immunol. 2021;11:625753.
  5. Immune Deficiency Foundation. What is PI? Accessed August 21, 2025. https://primaryimmune.org/understanding-primary-immunodeficiency/what-is-pi
  6. National Institute of Allergy and Infectious Diseases, National Cancer Institute. Understanding the immune system: how it works. US Department of Health and Human Services, National Institutes of Health; 2003.
  7. Data on file, Grifols.
  8. Perez EE, Orange JS, Bonilla F. et al. Update on the use of immunoglobulin in human disease: a review of evidence. J Allergy Clin Immunol. 2017;139(suppl 3):S1-S46.
  9. Siegel J. Immune globulins: therapeutic, pharmaceutical, cost, and administration considerations. Pharmacy Practice News. April 11, 2024. Accessed August 21, 2025. https://www.pharmacypracticenews.com/Monographs-and-Whitepapers/Article/04-24/Immune-Globulins-Therapeutic-Pharmaceutical-Cost-and-Administration-Considerations/73454