There are more than 300 disorders or conditions that are part of the group “primary immunodeficiency disease,” or PIDD. Each disorder negatively affects the function of the immune system, causing PIDD patients to be more at risk of experiencing illnesses and their frustrating symptoms.
The immune system fights off bacterial, viral, and fungal infections, but a missing or weakened immune system is not able to ward off infection too well. As a result, PIDD patients develop lengthy, often reoccurring issues, such as sinus infections, pneumonia, stomach bugs, ear infections, bronchitis, and more.
Allergies and asthma are both caused by an overreactive immune system, so a malfunctioning or even an absent immune system can often cause PIDD patients to exhibit symptoms that appear to be the same or similar to those of allergies or asthma. As a result, our team at Langford Allergy partners with local infectious disease specialists to diagnose and care for PIDD patients through individualized treatment plans.
What Are Signs of PIDD?
PIDD is a genetic issue, and most patients with PIDD are diagnosed in infancy or early childhood. But sometimes, patients present symptoms that appear as typical allergies or asthma and may not receive a diagnosis until they reach adulthood.
Signs of PIDD can include:
- Recurring, stubborn infections (ear infections, sinusitis, pneumonia, etc.)
- Failure to thrive (FTT) in childhood
- Autoimmune disorders
- Blood disorders
- Digestive issues
- Swollen lymph glands
- Recurring internal or external abscesses
- Family history of PIDD
While some patients can only exhibit a few signs of PIDD, such as recurring ear infections and delayed growth in childhood, others can exhibit more severe signs, such as having multiple autoimmune disorders, constant digestive problems, and more.
Types of PIDD
PIDD is made up of more than 300 primary immunodeficiency disorders. That’s not to say patients with PIDD have 300+ disorders; it just means they could have one or even multiple disorders from the PIDD group.
Just some of the types of PIDD include:
X-Linked and Autosomal Recessive Agammaglobulinemia
Patients with x-linked agammaglobulinemia (XLA) and autosomal recessive agammaglobulinemia (ARA) do not have the cells that produce antibodies, which causes them to easily develop infections in the ear, sinuses, lungs, skin, and digestive system.
Common Variable Immune Deficiency
Patients with common variable immune deficiency (CVID) don’t develop enough serum immunoglobins and antibodies, so they are more prone to infections.
Complement Deficiencies
Patients with complement deficiencies experience delays on the cellular level during the infection-fighting process. As a result, they are not able to ward off infections as well as others.
Hyper IgM Syndrome
Patients with hyper IgM syndrome (HIGM) have too little immunoglobin G and too much immunoglobin M. This imbalance makes them susceptible to recurring, stubborn infections.
IgG Subclass Deficiency
Immunoglobin G (IgG) is the main immunoglobin in the blood, but it is made of four subclasses. IgG subclass deficiency causes patients to have a deficiency with any one of the subclasses, which leads to recurring infections, especially respiratory infections.
Innate Immune Defects
A patient with innate immune defects has an immune system that cannot perform properly due to any number of defects in the system’s operation. As a result, the patient is susceptible to allergies, asthma, and a variety of infections.
NEMO Deficiency Syndrome
NEMO deficiency syndrome is a complex condition where genetic mutations in the NEMO (NF-kappa B Essential Modulator) gene cause deficiencies in several systems throughout the body. Patients with NEMO deficiency syndrome can suffer from severe bacterial infections anywhere in the body, including the lungs and skin.
Selective IgA Deficiency
Selective IgA deficiency is a condition where patients have a severely low amount of immunoglobin A (IgA) in the blood. Without the ability to produce proper amounts of IgA antibodies, patients often suffer from recurring infections and digestive issues.
Selective IgM Deficiency
Selective IgM deficiency is a deficiency in the production of immunoglobin M (IgM), which helps ward off infections. Patients tend to struggle to fight off infections in the lungs, ears, and sinuses.
Severe Combined Immune Deficiency
Severe combined immune deficiency (SCID) is considered one of the most serious PIDD types because it is a life-threatening condition. Patients with SCID do not have T cells and B cells that function properly. T cells help fight off “invaders” in the body, while B cells make the antibodies needed to remember an infection so that the body can fight it even better next time. Without the proper function of these two cells, patients are vulnerable to serious infections that their bodies cannot fight.
Specific Antibody Deficiency
Specific antibody deficiency is typically an issue in which the patient produces normal levels of immunoglobin G (IgG) but does not produce enough IgG antibodies to fight infections. As a result, the patient is susceptible to recurring infections in the ears, sinuses, and lungs.
WHIM Syndrome
In WHIM syndrome, the WHIM stands for Warts, Hypogammaglobulinemia (a disorder where the body does not produce enough antibodies), Infections, and Myelokathexis (a disorder of white blood cells). Patients with WHIM syndrome typically develop severe infections of the skin, ears, lungs, and sinuses, as well as other painful issues, usually in early childhood.
Wiskott-Aldrich Syndrome
Wiskott-Aldrich syndrome (WAS) is a genetic disorder that affects the immune system and bone marrow. Patients with WAS are prone to infections, eczema, and abnormal bleeding.
If you believe you may be suffering from a type of PIDD or your child may have PIDD, talk to Langford Allergy.
Dr. Langford and our team will discuss your history of infections, allergies, or asthma and will perform any necessary diagnostic testing to help us identify immunodeficiencies. If we diagnose PIDD, we will create an effective treatment plan.
Schedule a consultation with our team today: 478-787-4728
Langford Allergy delivers allergy, asthma, and immune deficiency care to patients throughout Middle Georgia, including:
- Macon, GA
- Warner Robins, GA
- Milledgeville, GA
- Forsyth, GA
Related articles:
Primary Immune Deficiency Diseases (PIDD) in Adults: What You Should Know