Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (CVID) is an immune system disorder characterized by low protective antibodies and an increased risk of infections.  In this condition, the immune system is weaker than usual. CVID is characterized by the following:

• Low levels of most or all of the immunoglobin [Ig] classes.
• Lack of B lymphocytes of plasma cells that can produce antibodies.
• Recurrent infections caused by bacteria.

Although the disease is usually diagnosed in adults, it can occur in children. 

Complications

CVID weakens the immune system. Due to the weakening of immunity, many health conditions can arise. Such complications include:

• Cancer
• Bronchiectasis
• Lung infections
• Enlarged lymphoid tissues
• Kidney problems
• Reduced liver function or failure
• Poor response to vaccines decreased in life expectancy
• Recurring infections, etc.

Diagnosis

The diagnosing of common variable immunodeficiency [CVID] is a difficult task. Therefore, the diagnosis of CVID is denoted by its symptoms. However, the diagnosis can be made using these criteria if the person:

• 4years and above.
• Shows a marked decrease in serum IgG levels [˂4.5g/L], IgA, IgM(occasionally).
• Lacks antibody immune response of protein antigen or immunization.
• Quantification of T cells and B cells by flow cytometry. It helps distinguish CVID from other immunodeficiency disorders such as multiple myeloma, chronic lymphocytic leukemia, X-linked agammaglobulinemia [1].
• Imaging studies such as CT scans of the chest help detect pulmonary abnormalities.
• Histological findings: histological changes noticed in lymph nodes are: atypical hyperplasia, granulomatous inflammation, reactive follicular hyperplasia [2].
• Other tests include Bronchoscopy, biopsy, endoscopy, etc.

Diagnosing CVID is not easy because of the diverse kinds of phenotypes seen in people. 

Treatment for CVID

There are many ways or methods of treating CVID. Treatments include:

• Regular Immunoglobulin (Gammaglobulin) Therapy

It is administered intravenously or by subcutaneous infusion with antibodies obtained from the fluid portion of the blood [gammaglobulin].

• Antibiotic Medications 

Antibiotics are used to treat various bacterial infections associated with CVID.

• Vitamin B12 Injections

CVID patients with irregularities in mal-absorption of vitamins are given B12 injection monthly.

• Surgical care: 

It is required to treat complications of CVID, such as in the case of chronic sinusitis. Endoscopic sinus surgery is performed in severe cases of hemolytic anemia, or autoimmune thrombocytopenia splenectomy is done. In the case of large lymph nodes, a biopsy is done to exclude malignancy or infection.

• Other symptomatic and supportive treatments include:

Genetic counseling for affected persons and their family members if their CVID is genetic. Furthermore, patients should be monitored for potential complications by undergoing periodic tests, including:

• Complete blood count [CBC] to detect lymphoma.
• Differential white blood cell counts to detect lymphoma.
• Annual thyroid examination and thyroid function testing.
• Annual pulmonary [lung] function testing, beginning about 8 to 10 years.
• Biopsy of enlarged lymphoid tissues.
• Other imaging techniques for assessment of granulomatous disease and gastrointestinal complications.

Patients affected with severely low levels of circulating platelets should not use aspirin since it may interfere with the ability of platelets to assist the blood-clotting process.  Also, people with CVID should avoid live virus vaccines because vaccine strains of viruses may cause diseases due to their weakened immune systems.

TIPS TO BOOST YOUR IMMUNE SYSTEM

• Eat a small portion of protein at every meal.

Certain nutrients take the lead in helping protect your body from billions of viruses, bacteria, and other germs.  Protein is an excellent example because the antibodies that help fight diseases are protein. Many protein-rich foods also contain other immune-boosting nutrients. For example, protein from beans, soy, and seafood contains zinc. Zinc helps to increase the production of infection-fighting cells like white blood cells. Other sources of protein include nuts like almonds and cashew.

•  Ensure to go for fruit ties and veggies rich in vitamins A, C, and E.

Vitamin A helps white blood cells chase infections away more effectively. It also plays a massive role in regulating the whole immune system, which can be derived from apples. Vitamin C improves iron absorption from plant-based foods. Like vitamin A, it helps ward off diseases from the immune system. Sources of vitamin C include:

• Pepper.
• Papaya.
• Broccoli.
• Citrus fruits, such as:
• Lemons.
• Oranges.
• Grapefruits.

As for vitamin E, it battles flu and upper respiratory infections. Vitamin E is in nuts, seeds, and green vegetables. Taking multivitamins or mineral supplements may sometimes help reduce the effects of common variable immunodeficiency [CVID], but high doses may cause imbalances and suppress your immune system.

• Regulate the rate at which you take vitamin D.

Only approximately 50 nmol/L of vitamin D is generally enough to maintain overall health. Less than 30 nmol/L may be too low for some individuals. Conversely, vitamin D may be too low for some individuals. Vitamin D can boost immune response, while too low levels may be linked to increasing colds and flu.  Some sources of vitamin D include: Eggs, Cereals, Oranges, Cheese, Beef liver, Soy milk

• Cook with olive and canola oils.

Olive and canola oils contain healthy fats, which act as lubricants to cells. As a result, they improve the communication between cells, promoting immune function.  WARNING: Don’t consume too many omega-6 fats.

• Daily exercises.

Take a 10-minute walk, few times a day. 20 to 30 minutes of daily physical activities help in boosting your immune system. It gets antibodies and white blood cells to move fast through your body to detect infections.

• Reduce your stress levels.

You can restrain or eliminate stress by:

• Listening to music.
• Reading journals.
• Listening to scriptural or biblical messages.
• Associating more with people.
• Feeling excited, joyful, and happy.

Check off the rate at which you get drunk. Your body produces specific amounts of alcohol for certain metabolic requirements. In addition, high levels of alcohol weaken your immune system.  Discuss with your doctor how you can help yourself by reducing your intake of alcohol. It is medically advisable that you even quit drinking. CVID LIFE EXPECTANCY The life expectancy of common variable immunodeficiency has improved, increasing from 12 years to 50 years of living with the condition. Once managed effectively, a patient can live a full, healthy life.   

Symptoms

CVID is caused by a diversity of different genetic abnormalities that leads to a defect in the ability of immune cells to produce exact amounts of all types of antibodies. Only a few of these fat faults have been identified, and the cause of most cases of CVID is unknown. 

Many CVID patients carry a DNA variation called polymorphies is a gene known as TACL. Although this genetic abnormality has increased the rate of developing CVID, it cannot on its cause CVID.

CVID comes up as a result of defects in the genes involved in the immune system. Due to the defect, the body would produce abnormally low amounts of proteins called immunoglobulins, among which is immunoglobulin G [IgG]. When levels of IgG are low in the blood, the body finds it difficult to fight infections. Low levels of IgG in the blood depict a weakened immune system.

IgA deficiency usually occurs alone, but in certain situations, it may occur before CVID develops or occur in CVID patient’s family members. 

In most cases, this genetic mutation and CVID symptoms develop without any obvious cause. About 10% of CVID cases are hereditary. 

Causes

The signs of CVID may vary from one person to another. They can also range from mild and severe. 

These indications may include any of the below:

• Chronic cough.
• Breathing problems.
• Diarrhea.
• Recurring lung infections, such as pneumonia and asthma.
• Loss of weight.
• Ear infections.
• Frequent sinus infections.
• Bronchiectasis [a chronic form of bronchitis].
• Absorption problems.
• Reduced liver function.
• Impaired blood flow to the liver.
• Reduced levels of blood cells or platelets.
• Enlarged spleen.
• Swollen glands or lymph nodes.
• Painful swollen joints in the knee, ankle, elbow, or wrist.

FAQ

Is CVID life-threatening?

With ongoing treatment, many individuals with CVID live active and fulfilling lives. However, in some cases, complications of CVID (such as lung damage or cancer) may affect life expectancy. These complications appear over time and may become a threat to one’s life, but the process often takes years.

How to know if you are immunocompromised?

Indications that point out a weak immune system are apparent. For example, the American Academy of Allergy Asthma and Immunology reports signs of a possible immune deficiency in adults to include:

• Having more than four episodes of ear infections in one year.
• Developing pneumonia twice during one year.
• Suffering from chronic sinusitis.
• Over three episodes of bacterial sinusitis annually.

What are the three common autoimmune disorders?

The three autoimmune diseases are:

• Type 1 diabetes: Here, the immune system attacks cells in the pancreas that make insulin.
• Rheumatoid arthritis: This type of arthritis causes swelling and deformities in the joints.
• Lupus: It attacks body tissues, kidneys, the lungs, kidneys, and skin.

What are the different types of immunodeficiency?

The main types of primary immune deficiency diseases include:

• Autoimmune lymphoproliferative syndrome [ALPS].
• APS-1.
• CARD-9 deficiency and other symptoms of susceptibility to candidiasis.
• Chronic granulomatous disease [CGD].
• Common variable immunodeficiency [CVID].
• Congenital neutropenia syndromes.
• CTLA-4 deficiency.
• DORK-8 deficiency.

What is the main cause of immunodeficiency?

It is known that when most people hear of immunodeficiency, the first thing that comes to mind is the thought about HIV or AIDS. However, there are many causes of immunodeficiency. The most common causes include HIV, malnutrition, and unsanitary conditions.

 

 

References:

1. msdmanuals
2. Emedicine.Medscape