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Immunodeficiency, immunocompromised, and autoimmune disease are all terms that describe the body’s immune system and its reduced ability or inability to fight infection or disease. But the terms are not always interchangeable. Here’s what each means.
What are Immunodeficiency Disorders?
Immunodeficiency disorders are dysfunctions of the immune system that can prevent a person’s body from defending itself against disease or infection.
This could mean that the body’s immune system is not making enough immune cells or antibodies to fight off foreign invaders, or that the body’s immune response is absent.
As with many medical conditions, immunodeficiency can range from mild to severe. In mild cases, an immunodeficient person may not even realize it, according to Elizabeth M. Younger, assistant professor of pediatrics at the Johns Hopkins School of Medicine in Maryland. This is because you can be asymptomatic with some of the milder forms.
There are two types of immunodeficiency disorders, depending on the British Society for Immunology: primary and secondary. Primary immune deficiencies are those that are genetic or hereditary, while secondary immune deficiencies develop due to environmental factors and are more common than primary immune deficiencies.
Primary immunodeficiency
There are more than 400 types of primary immunodeficiencies, according to the Centers for Disease Control and Prevention (CDC). According to American Academy of Allergy, Asthma and Immunology, while symptoms of less severe disorders may not appear until adulthood. For example, the median age of onset of Common Variable Immune Deficiency (CVID) is 24, according to a 2014 review published in Allergo Journal International.
The signs of a primary immunodeficiency disorder are as follows:
- Frequent or recurring infections, which may include sinus infections, ear infections, bronchitis, pneumonia, skin infections or candidiasis (fungal infection caused by a type of yeast called Candidiasis)
- Persistent infections
- Bacterial infections that do not respond well to antibiotics
- Infections that require hospitalization, such as sepsis
Treatments vary depending on the type of immunodeficiency disorder and the particular infection.
The most common primary immune deficiency is selective IgA deficiency (SIgAD), according to the Immunodeficiency Foundation. People with selective IgA deficiency lack immunoglobulin A, or IgA, which is an antibody found in the lining of the respiratory and digestive tracts, according to Johns Hopkins Medicine.
SIgAD most commonly occurs in people of European descent, according to the Cleveland Clinic. For some, SIgAD can cause serious illness, but many people with the disorder don’t, Younger said. Some people don’t even notice any symptoms.
The vast majority of them have no idea that their IgA levels are very low or completely absent. “They’re walking around, completely asymptomatic, and that’s never a problem at all,” Younger said.
However, deficiency increases a person’s vulnerability to infections, just like other primary immunodeficiency disorders.
Secondary immunodeficiency
Secondary immunodeficiency disorders are more common than primary immunodeficiency disorders, according to a 2009 review published in the Journal of Allergy and Clinical Immunology, and develop due to environmental factors. For example, secondary immunodeficiency disorders can be caused by HIV infection; certain drugs, such as drugs to prevent rejection of transplanted organs; or treatment with chemotherapy. Malnutrition is the most common cause of immunodeficiency worldwide, as calorie and micronutrient deficiencies can impair the immune response.
Symptoms of a secondary immunodeficiency disorder include frequent infections and occasional opportunistic infections, which tend to occur more frequently in people with weakened immune systems. For example, candidiasis is a fungal infection caused by the Candidiasis yeast. Yeast can live on your skin and in your body without causing problems, but if you have a weakened immune system it can get out of hand and cause infections, according to the doctor. CDC.
Diagnosis & treatment
To diagnose immunodeficiency, a doctor will likely do a physical exam, get a complete patient history, and perform various tests, depending on the patient. Manuel Merck. Tests can include blood tests, skin tests, a lymph node or bone marrow biopsy, and sometimes genetic testing to identify a genetic mutation that could be causing the disorder.
The type of treatment depends on the type of immunodeficiency disorder. According to National Jewish Health, treatment may include antimicrobial therapy for infections, vaccinations, or specialized therapies to replace or supplement cells of the immune system.
The prognosis for people with immunodeficiency disorders also varies. Many people with primary immunodeficiency require continued treatment with antibiotics and antifungals to avoid infection. In the case of some secondary immunodeficiencies, treatment of the primary cause can effectively resolve the immunodeficiency, depending on the British Society for Immunology; an example is malnutrition.
Is immunodeficiency the same as autoimmune disease?
Immunodeficiency disorders and autoimmune diseases are not exactly the same. An immunodeficiency is a deficiency in the immune system, while an autoimmune disease occurs when the immune system attacks healthy cells, tissues, and organs in the body.
However, studies have shown that there is often a link between immune deficiencies and autoimmune diseases. “Patients with certain types of immunodeficiency have a propensity for autoimmune diseases,” Younger said, adding that it is common for autoimmune disease to develop after diagnosis of immunodeficiency. “They absolutely go together,” she said.
People with primary immunodeficiency are more likely to have autoimmune disease, certain blood disorders and cancers, according to the CDC, because their immune systems just aren’t working as effectively to protect them from these health issues.
There are more than 80 recognized autoimmune diseases, including type 1 diabetes, inflammatory bowel disease and rheumatoid arthritis, according to National Institute of Allergies and Infectious Diseases. Medicines prescribed to treat autoimmune diseases usually work to suppress the immune system in order to slow it down or prevent it from attacking the body and in doing so, increase the risk of other infections. So there is some overlap: a person may be immunodeficient or immunocompromised as a result of treatment they are receiving for an autoimmune disease.
Is immunodeficiency the same as being immunocompromised?
Immunocompromised is a general term to describe a person with a weakened immune system. People who are immunocompromised have a higher risk of getting sick – because their immune systems have a harder time fighting bacteria, viruses, and other invaders – than someone with a healthy immune system.
People with immunodeficiency or autoimmune disease are considered to be immunocompromised, but other factors can also make a person immunocompromised, such as cancer, cancer treatments, metabolic disorders or advanced age, depending on University of Texas MD Anderson Cancer Center. Transplant patients and smokers are also immunocompromised, according to Penn medicine.
Immunodeficiency and COVID-19
Anyone with an immunodeficiency may be at increased risk of developing serious complications if they contract COVID-19, according to the CDC. People with cancer; chronic diseases, such as chronic diseases of the liver or lungs; or autoimmune diseases may also be at increased risk. The research supports this, as a study published in late 2020 in the Journal of Allergy and Clinical Immunology found that adults with primary immunodeficiencies and symptomatic secondary immunodeficiencies had higher morbidity and mortality from COVID-19.
Anyone who is immunocompromised should be vaccinated against COVID-19, said Dr. Aaron E. Glatt, chair of the department of medicine and chief of infectious diseases at Mount Sinai South Nassau and spokesperson for the Infectious Diseases Society of America. But the vaccine is not a perfect solution. “We understand that people who are immunocompromised are not as sensitive to the vaccine, which is a major concern,” said Glatt.
Research from the University of Pittsburgh School of Medicine has found that certain groups of immunocompromised patients are unable to produce enough antibodies to protect them from COVID-19, and therefore still have an increased risk of infection even after the vaccination. This study has not been peer reviewed and is available on the preprint server MedRxiv.
People with weakened immune systems should be very careful about protective measures, including social distancing and wearing a mask, Glatt said.
Additional resources
This article is for informational purposes only and is not intended to offer medical advice.
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