What is Primary Immunodeficiency?

If you or a loved one has received a diagnosis of primary immunodeficiency (PI), you may be feeling confused or overwhelmed. Very likely, you will have many questions. Octapharma is here to help, offering information, support, and advanced PI therapy.

What is primary immunodeficiency?

Primary immunodeficiency is a relatively rare disorder that weakens the immune system, allowing infections and other chronic health problems to occur more easily, more frequently, and to last longer. There are more than 350 recognized types of primary immunodeficiency disease, in which part of the body’s immune system is missing or functions improperly. PI is caused by hereditary or genetic defects and can affect anyone, regardless of age or gender. While the types of the disease may differ, they all share one common feature: each is the result of a defect in at least one of the functions of the body’s normal immune system.1 The most common types of primary immunodeficiency result from the inability to make a very important type of protein called an antibody, which protects your body against infections.2

You may hear PI referred to by different names, including:

  • Primary immunodeficiency
  • Primary immune deficiency
  • Primary humoral (humoral means having to do with antibodies) immunodeficiency
  • Primary immunodeficiency disorder
  • Primary immunodeficiency disease

Approximately 250,000 people in the U.S. are diagnosed with PI; many more may go undetected.3 While at present there is no cure for primary immunodeficiency, patients can manage their disease through various therapies and, with proper medical care, go on to live full and independent lives.

How PI can affect you

If you have a common type of PI—where you are missing antibodies in your immune system—your body is less able to fight bacteria and viruses that cause infections. As a result, you may experience repeated infections, infections that won’t go away, unusual infections, and severe infections.1 People with PI live their entire lives more susceptible to these infections—enduring recurrent health problems and often developing serious and debilitating illnesses.

Signs and Symptoms of PI

One of the most common signs of PI is having infections that can appear in a variety of areas, including your:

  • Skin
  • Sinuses
  • Throat
  • Ears
  • Lungs
  • Brain or spinal cord
  • Urinary or intestinal tracts1

PI signs and symptoms can also include:

  • Frequent pneumonia
  • Bronchitis
  • Inflammation of internal organs
  • Digestive problems such as cramping and loss of appetite
  • Autoimmune disorders including lupus, rheumatoid arthritis, and type 1 diabetes1

Talk to your healthcare provider about any infections and other symptoms you may develop. You and your healthcare provider can team up to create an effective PI treatment plan.

Living with PI

Having PI can be isolating and frustrating. You have to face and overcome daily challenges when living with the disease. Keeping informed can help you succeed. Staying connected to other PI patients, the PI community, and beyond can also do wonders in developing and maintaining a positive spirit, attitude, and lifestyle. That’s why Octapharma developed the IgCares patient support program. This innovative program is designed to provide support for adults with PI at every step of their journey – minimizing hassle, improving quality of life, empowering through education, building community, and advancing research and the treatment of the disease.

Learn more about IgCares and join the program at igcares.com, or call 1-833-382-7686.

Connect and Learn More

There are many resources available to you to help you become more educated about PI, connect with others, and become an active member of the PI community:

World PI Week
2020 Plans
World PI Week offers an opportunity to inform and educate health policy makers, schools and families, and the general public about primary immunodeficiencies (PI) to drive the earliest possible diagnosis and optimal treatment.

The Immune Deficiency Foundation (IDF)
Dedicated to improving the diagnosis, treatment, and quality of life of people with PI.

The Jeffrey Modell Foundation
A global resource for the most up-to-date information and support for people with PI.

The Foundation for Primary Immunodeficiency Diseases (FPID)
Supporting PI education, early diagnosis, counseling, therapy, and research.

International Patient Organisation for Primary Immunodeficiencies
Dedicated to improved awareness, access to early diagnosis, and optimal treatments for PI patients worldwide.


Exclusive educational materials to get you up to speed and keep you informed about all aspects of living well while living with PI. Join the IgCares patient support program to access all the information.

  1. Immune Deficiency Foundation (IDF). About Primary Immunodeficiencies. https://www.primaryimmune.org/about-primary-immunodeficiencies. Accessed April 25, 2019.
  2. Cutaquig Full Prescribing Information. Paramus, NJ: Octapharma; rev October 2021.
  3. Immune Deficiency Foundation (IDF). “Primary Immunodeficiency Diseases in America: 2007” National Survey. May 1, 2009. Pages 17, 45.