What Are The Primary Disorders Of The Immune System?

The immune system is vital for survival, and it provides all the required support to fight infections and diseases. But what if your immune system is damaged? Several factors can cause immune system disorders, but do you know that some people have weak immune systems by birth which is known as primary immunodeficiency. This means they are more likely to get infections and diseases since their immune system does not fight back enough. Let us learn about the primary disorders of immune system.

There are more than 200 forms of primary immunodeficiency disorders. Usually, the immune system works to attack any kind of infections or toxins that try to invade our body. However, people with primary immunodeficiency disorders are more likely to suffer from various infections due to certain defects in the immune system.

Signs Of Primary Immunodeficiency:

Although there are many types and several other causes of primary disorders of the immune system, most of them share similar signs. They are:

• Repetitive infections of the ear, sinus, lungs, etc.
• Prolonged infections
• Lack of growth or weight gain in infants
• Infections affecting the internal organs requiring frequent hospitalisation
• Chronic diarrhoea
• Autoimmune disorders
• Blood disorders

List Of Primary Immunodeficiency Disorders:

Here is a list of 7 prominent primary immunodeficiency disorders:

1. Chronic Granulomatous Disease:

The chronic granulomatous disease occurs when a component of white blood cells called phagocytes cannot kill the invading bacteria and fungi. As a result, the person will be vulnerable to infections. Alteration in one or two genes and defects in enzyme NADPH oxidase are said to be the two main causes of these disorders of immune system.

2. Severe Combined Immunodeficiency:

This is a classic example of primary immunodeficiency disorder that is present right from birth. It is also referred to as the Bubble boy disease. Children with this disorder are highly vulnerable to bacterial, viral and fungal infections.

Primary immunodeficiency disorders usually have symptoms such as chronic diarrhoea, failure to thrive, recurrent infections and skin rashes. Some people might also experience neurological deficits. This is an emergency disorder since it leads to death, and a stem cell transplant might be helpful.

3. Hyper IgE Syndrome:

Hyper IgE syndrome is another combined immunodeficiency disorder that shows symptoms like skin and bone infections. It occurs due to changes in genes that ultimately affect the way the body recognises staphylococcus bacteria.

It is a rare disorder caused by changes in the STAT3 and DOCK8 genes. These genes generate signals to make the immune system respond to pathogens. While the amount of these genes remains normal, its function will be inadequate to fight the invading pathogen. 

4. X-linked Agammaglobulinemia:

X-linked agammaglobulinemia is a disorder of immune system characterised by defects in the B cells of white blood cells. This defect deprives the body of synthesising mature B cells and plasma cells. Ultimately, people with this disorder lack immunoglobulin and the ability to produce antibodies to fight infections.

Although there is no cure for defects in genes, immunoglobulin injections help manage them. These injections contain antibodies that act on behalf of the body's natural antibodies to act against certain infectious agents. People with this condition are not supposed to receive live vaccines.

5. Common Variable Immunodeficiency:

Common variable immunodeficiency includes conditions that change how a person's body produces antibodies to fight infections. People with common variable immunodeficiency are highly prone to recurrent bacterial infections. The cause for these conditions is not well known, and most of the patients discover that they have this defect only during their adulthood.

It is the most common primary immunodeficiency disorder consisting of various clinical and immunological features. The main treatment option for CVID is immunoglobulin replacement therapy. The health care team might suggest a stem cell transplant in severe cases.

6. Selective IgA Deficiency:

Selective IgA deficiency affects one in 600 people, and it is usually linked with autoimmune or allergic diseases. The exact genetic cause is unknown. However, it is said that people with this condition lack one type of antibody known as Immunoglobulin A. 

Common symptoms include allergies, infections and autoimmunity that results in celiac disease. 

Most people do not require treatment. However, symptoms will be treated with antibiotics, steroids, supplements and immunoglobulin replacement therapy if needed. If celiac disease is confirmed, following a gluten-free diet will be helpful.

7. Disorders Of Immune Dysregulation:

These primary immunodeficiency disorders comprise autoimmune diseases. They occur due to dysregulation in the immune system as a whole. White blood cells and their components play a significant role in the immune response. In immune dysregulation disorders, the components of white blood cells will be present in the right amount, but they do not function. This results in a problem where the immune system is hyperreactive to its own cells, leading to an autoimmune disorder. 

Identify The Altered Immune Responses At Right Time:

Right now, there is no permanent cure for primary immunodeficiency. Certain treatment options help reduce the severity of the condition.  

Reach out to your health care provider If you suffer from recurring infections, it would be best to see your doctor irrespective of your diagnosis. Your healthcare practitioner might provide a referral to a clinical immunologist to get proper treatment. Tell your immunologist if anyone in your family is suffering from PID.