How to Manage Autoimmune Disease Risk With Primary Immunodeficiency
Managing Primary Immunodeficiency

How to Manage Your Risk of Autoimmune Disease When You Have Primary Immunodeficiency

How to Manage Your Risk of Autoimmune Disease When You Have Primary Immunodeficiency
iStock
By
Published on August 13, 2025
by

Primary immunodeficiency (PI) not only increases your risk of frequent and serious infections, but can also make you more likely to develop autoimmune diseases — conditions where the immune system mistakenly attacks healthy cells in the body.

Autoimmune conditions such as lupus, rheumatoid arthritis, type 1 diabetes, and others have been linked to different forms of PI. One study found that 26% of people with common variable immune deficiency had at least one autoimmune condition, and many had more than one.

“When regulation of the immune system is off, it gets confused and starts attacking different parts of the body,” says Mark Ballow, MD, a professor of pediatrics in the division of allergy and immunology at the University of South Florida in St. Petersburg. “It doesn’t know that it’s not supposed to do that, and that’s why autoimmunity can develop.”

Understanding your risk of developing an autoimmune disorder can help you stay one step ahead, so you and your care team can make informed, timely decisions together. Keep reading to learn how.

The Link: How PI Can Increase Your Risk of Autoimmune Diseases

To understand how PI can raise your risk of autoimmune diseases, it’s helpful to know a bit more about how the immune system works. In immunology, there is a concept called self-tolerance, which is the immune system’s ability to differentiate between its own cells and foreign bodies, such as bacteria.

 If self-tolerance fails, says Dr. Ballow, the body may attack its own healthy cells, which is known as autoimmunity. The immune system abnormalities in PI can cause these disruptions, potentially causing autoimmune disease.

Autoimmune problems can affect both children and adults with PI. They’re especially common in certain types of PI, including common variable immune deficiency, complement deficiencies, Good syndrome, hyper immunoglobulin M syndrome, idiopathic T cell lymphocytopenia, selective immunoglobulin A deficiency, and Wiskott-Aldrich syndrome.

While there’s no proven way to prevent autoimmune diseases in people with PI, staying proactive about your healthcare may make a difference. “We can’t really predict which patients will develop autoimmune disease,” says Ballow. “However, if we do genetic testing and we define a genetic abnormality ... we can follow you a little more closely and perhaps catch an autoimmune process a little earlier.”

Common Autoimmune Diseases Linked to PI

When you have PI, autoimmune diseases can appear in many different ways, depending on the type of PI you have and how your immune system is affected. Autoimmune problems can even be the first sign of the condition.

In fact, data from the Immune Deficiency Foundation (IDF)’s 2023 National Patient Survey

 found that nearly one in three people with PI were diagnosed with an autoimmune condition before they were diagnosed with PI. Unfortunately, this often led to delays in diagnosis — sometimes by more than 10 years. But greater awareness of this overlap can help you and your doctor recognize the full picture sooner and start appropriate care.

Some of the most common autoimmune diseases seen in people with PI include:

  • Blood-related autoimmune diseases (autoimmune cytopenias), such as autoimmune hemolytic anemia — where the immune system destroys red blood cells, causing fatigue and weakness — and Evans syndrome, a rare disorder where the body attacks both red blood cells and platelets.
  • Digestive system autoimmune diseases, such as Celiac disease, which is an immune reaction to gluten that damages the small intestine, as well as Crohn’s disease and ulcerative colitis — conditions that cause chronic inflammation in the digestive tract.
  • Skin, joint, and connective tissue autoimmune conditions, such as lupus, a chronic disease that causes inflammation and damage to various parts of the body, and rheumatoid arthritis, which is an immune-driven attack on the joints that causes pain, stiffness, and swelling.
  • Endocrine (hormone-related) autoimmune disorders, such as Hashimoto’s thyroiditis — an immune attack on the thyroid gland that leads to an underactive thyroid — and type 1 diabetes, in which the immune system destroys cells that make insulin in the pancreas.
  • Autoimmune lung diseases, such as granulomatous lymphocytic interstitial lung disease, which is a lung condition seen in some people with PI that causes inflammation and scarring, and interstitial lung disease, a group of conditions that make lung tissue stiff and reduce breathing ability.

Tips to Reduce Your Risk of Autoimmune Disease When You Have PI

While there’s no guaranteed way to prevent autoimmune disease, there are important steps you can take to manage PI and stay aware of any changes in your health:

  • Monitor your health. Watch for symptoms that might signal an autoimmune disease, such as bruising, digestive issues, fatigue, joint pain, or frequent infections.
  • Get regular checkups. “We see patients back every six months — or sooner if they develop new symptoms,” says Ballow.
  • Talk to your doctor about genetic testing. “If we define a genetic abnormality, we can follow it more closely and perhaps catch an autoimmune process a little bit earlier than usual,” says Ballow. Some genetic findings may even open the door to precision medicine, which is a targeted treatment approach to help manage or prevent autoimmune complications.
  • Stay connected with a clinical immunologist. “It’s so important to see a clinical immunologist,” says Ballow. “They can really try to understand your immune deficiency.” Both the IDF and Jeffrey Modell Foundation have PI clinical finder tools to help you find a provider.
  • Be your own advocate. “Patients have to advocate for themselves,” says Ballow. “There are a lot of undiagnosed patients with primary immune deficiency” and potentially autoimmune diseases, too. While it’s easier said than done, you can advocate for yourself in small but meaningful ways, such as writing down three questions before your next appointment and making a point of getting answers.
  • Practice good habits. Prioritize sleep, balanced nutrition, regular movement, and stress management to help keep your immune system strong.
  • Lean on trusted organizations. Groups such as the IDF and the International Patient Organization for Primary Immunodeficiencies provide education, resources, and supportive communities to help you manage life with PI.
  • Stay positive and proactive about your care. “I would be optimistic, because we have lots of new ways to treat autoimmunity,” says Ballow. Working closely with your specialists can help you catch and manage autoimmune issues earlier, reducing their impact on your health and quality of life.

The Takeaway

  • Autoimmune diseases are more common in people with primary immunodeficiency than you may realize and can sometimes be the first sign of PI.
  • While you can’t always prevent autoimmune conditions, regular monitoring and genetic testing can help detect them early and open the door to new treatment options.
  • Advocacy, education, and a strong care team, including a clinical immunologist, are essential to managing PI and staying ahead of future risks.
EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
Resources
  1. Fischer A et al. Autoimmune and Inflammatory Manifestations Occur Frequently in Patients With Primary Immunodeficiencies. The Journal of Allergy and Clinical Immunology. November 2017.
  2. Sullivan K. Patient & Family Handbook for Primary Immunodeficiency Diseases [PDF]. Immune Deficiency Foundation.
  3. Autoimmunity. Immune Deficiency Foundation. 2019.
  4. 2023 National Patient Survey. Immune Deficiency Foundation.
Meet Our Experts
See Our Editorial PolicyMeet Our Health Expert Network
jon-stahlman-bio

Jon E. Stahlman, MD

Medical Reviewer
Castle Connolly Top Doctor

Jon E. Stahlman, MD, has been a practicing allergist for more than 25 years. He is currently the section chief of allergy and immunology at Children’s Healthcare of Atlanta's Scottish Rite campus and the senior physician at The Allergy & Asthma Center in Atlanta. He served as the president of the Georgia Allergy Society, has been named a Castle Connolly Top Doctor, and was listed as a Top Doctor by Atlanta magazine. His research interests include new therapies for asthma and allergic rhinitis as well as the use of computerized monitoring of lung function.

He received his bachelor's and medical degrees from Emory University. He completed his pediatric residency at Boston Children’s Hospital and his fellowship in allergy and clinical immunology at Harvard University’s Boston Children’s Hospital and Brigham and Women’s Hospital. After his training, Dr. Stahlman conducted two years of clinical research at Boston Children’s Hospital and was part of the faculty at Harvard Medical School, where he taught medical students and allergy and immunology fellows.

Stahlman is board-certified and recertified in allergy and clinical immunology. He served as a principal investigator on phase 2 through 4 studies that are responsible for most of the U.S. Food and Drug Administration–approved therapies for allergies and asthma available today.

Outside of the office, he centers his interests around his wife and three daughters, coaching soccer for many years, and his hobbies include cycling and triathlons.

See full bio

Susan Jara

Author

Susan Jara is a health communications strategist and writer with more than 15 years of experience transforming complex medical information into clear, accurate, and engaging content for diverse audiences of patients and caregivers. She specializes in patient education, health literacy, and SEO-driven content strategy, with expertise across chronic disease, mental health, addiction, arthritis, autoimmune conditions, and wellness.

Susan holds a bachelor’s degree in journalism and media studies from New York University’s Gallatin School of Individualized Study. Her career includes leadership roles at the Global Healthy Living Foundation and Health Monitor Network, where she developed multichannel health content across web, email, podcasts, video, social media, and print. Susan's work reaches millions of readers each year, and she collaborates with leading healthcare providers, researchers, advocacy groups, and industry partners to create resources that reach millions of readers each year.

See full bio
See Our Editorial PolicyMeet Our Health Expert Network