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PRIMARY IMMUNODEFICIENCY DISEASE (PI)

Primary immunodeficiency can take a real toll on daily life—causing frequent or chronic infections, persistent fatigue, and ongoing disruptions to your overall well-being. Wanting relief is natural, but it’s also important to feel confident and informed about your treatment options.

 

At Advanced Allergy, Asthma & Immunology, we take a thoughtful, patient-centered approach to care. We guide you through your infusion therapy options, address your concerns, and tailor treatment to your needs.

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Our goal is simple: to help you reduce infections, regain energy, and improve your quality of life—so you can start feeling like yourself again.

 

WHAT IS PRIMARY IMMUNODEFICIENCY DISEASE (PI)?

Primary immunodeficiency (PI) refers to a group of disorders in which part of the immune system is missing or does not function properly. As a result, the body has a reduced ability to fight infections.

 

These conditions are typically genetic and may be inherited. While some are present from birth, symptoms can appear later in childhood or even adulthood. PI can affect individuals of any age, gender, or background. There are hundreds of identified types—over 400—and new forms continue to be discovered.

 

In many cases, PI involves a problem with antibodies, which are proteins in the blood that help the body recognize and fight infections.

Common signs and symptoms may include:

• Frequent or chronic infections (such as sinus, ear, lung, skin, or gastrointestinal infections)

• Infections that are unusually severe, difficult to treat, or require hospitalization

• Enlarged lymph nodes, spleen, or liver

• Inflammation of blood vessels

• Poor growth or weight gain in infants (failure to thrive)

• Ongoing digestive issues, such as chronic diarrhea

• Autoimmune conditions (when the immune system attacks the body’s own tissues)

 

A family history of recurrent or severe infections may increase the likelihood of a primary immunodeficiency.

In addition to infections, individuals with PI may have a higher risk of autoimmune disorders, certain blood conditions, and some cancers.

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Early diagnosis is important. Testing—such as blood work and genetic evaluation—can help identify the condition, guide treatment, and reduce the risk of long-term complications.

 

TYPES OF PRIMARY IMMUNODEFICIENCY (PI)

There are hundreds of primary immunodeficiencies recognized by the International Union of Immunological Societies. Some of the more common deficiencies we see include:

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Specific Antibody Deficiency (SAD)
Specific Antibody Deficiency occurs when the immune system is unable to produce effective antibodies against certain bacteria, even though overall antibody levels may appear normal. This leaves the body less protected—particularly against bacteria with protective sugar (polysaccharide) coatings. Individuals with SAD often experience frequent sinus, ear, or lung infections, especially when the immune system is not mounting a strong response.

 

Common Variable Immune Deficiency (CVID)
Common Variable Immune Deficiency is one of the more frequently diagnosed primary immunodeficiencies and can affect both children and adults. It is characterized by low levels of key antibodies, leading to recurrent infections of the sinuses, lungs, and airways. Some individuals may also develop more serious complications such as pneumonia or chronic lung damage (bronchiectasis). In addition to infections, CVID can involve digestive issues, enlarged lymph nodes or spleen, joint pain, and an increased risk of autoimmune conditions and certain cancers.

 

IgG Subclass Deficiency
Immunoglobulin G (IgG) is the most abundant antibody in the body and plays a critical role in fighting infections. It is divided into four subclasses, each with a specific function. In IgG subclass deficiency, one or more of these subclasses are low or not functioning properly. This can lead to recurrent infections, particularly of the ears, sinuses, lungs, or gastrointestinal tract. It is most commonly recognized in children and may occur alongside other immune deficiencies.

 

Selective IgM Deficiency
Selective IgM Deficiency is a rare condition in which the body has low levels of IgM, an antibody that plays an important role in early immune response. Some individuals may have no symptoms, while others experience frequent or severe infections. The severity and presentation can vary widely, and in some cases, this condition may be associated with other immune system disorders.

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INFUSION THERAPY AND OTHER TREATMENTS FOR PRIMARY IMMUNODEFICIENCY DISEASE (PI)

Primary immunodeficiency (PI) disorders affect the body’s ability to defend itself against bacteria, viruses, fungi, and even abnormal cells such as cancer. As a result, individuals with PI may experience frequent or unusual infections and, in some cases, an increased risk of certain cancers. Additionally, up to 25% of individuals with immunodeficiency conditions may also develop autoimmune disorders, where the immune system mistakenly attacks the body’s own tissues—further complicating overall health.

Effective treatment options are available, and many patients experience meaningful improvement with the right care.

At Advanced Allergy, Asthma & Immunology, we offer a range of advanced biologic and immunotherapy treatments designed to support and strengthen the immune system. For patients who have not responded adequately to oral medications, infusion therapy can provide an effective next step to help reduce infections and improve quality of life.

We provide intravenous immunoglobulin (IVIG) therapy for patients with PI. IVIG is administered through a vein in the arm and is a well-established treatment that is safe, reliable, and generally well tolerated. This therapy works by supplying the body with the antibodies it needs to better fight infections.

 

IVIG and SCIG therapies offered at Advanced Allergy, Asthma & Immunology include:​

• Aceniv

• Alyglo

• Bivigam

• Gammagard Liquid

• Gamunex-C

• Hyqvia

• Octagam

• Panzyga

• Privigen

• Yimmugo

 

Because each patient’s condition is unique, certain medications may be more appropriate depending on age, diagnosis, and treatment goals.

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SIDE EFFECTS OF IVIG TREATMENT FOR PRIMARY IMMUNODEFICIENCY DISEASE (PI)

While IVIG therapy is generally safe and well tolerated, all treatments carry some degree of risk. Each patient may respond differently, and side effects can vary based on individual factors.

Common, typically mild side effects may include:

• Headache

• Chills

• Fever

• Flushing

• Muscle or joint aches (flu-like symptoms)

• Fatigue

• Nausea or vomiting

• Abdominal discomfort

• Skin rash

• Wheezing

It is not uncommon for these symptoms to occur during or shortly after an infusion. In many cases, they are temporary and resolve within 24 to 48 hours.

If side effects last longer than 48 hours, worsen over time, or become concerning, you should contact your healthcare provider. More severe symptoms—such as difficulty breathing, signs of an allergic reaction, or unusual changes in blood counts—require immediate medical attention and may warrant evaluation in an emergency room.

Our team closely monitors patients throughout treatment to help minimize side effects and ensure your safety at every step.

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WHAT TO EXPECT FROM IVIG INFUSION THERAPY FOR PRIMARY IMMUNODEFICIENCY DISEASE (PI)

If you are receiving immunoglobulin treatment for primary immunodeficiency (PI), it is important to understand what to expect. In addition to potential side effects, your experience with treatment can vary based on your specific condition, age, and dosage. We encourage open discussion so you feel informed and prepared throughout your care.

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At Advanced Allergy, Asthma & Immunology, patient comfort and safety are central to everything we do. Each infusion is carefully supervised by a skilled nurse with an on-site physician to ensure the treatment is well tolerated.

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While some patients notice improvement early, IVIG or SCIG therapy often takes time to reach its full benefit. Our team is here to assist you through each step and ensure your treatment experience is as comfortable and effective as possible.