Pediatric common variable immunodeficiency (CVID)

What is pediatric common variable immunodeficiency (CVID)?

Common variable immunodeficiency (CVID) is an antibody deficiency that leaves a child’s immune system vulnerable to recurrent infections, autoimmune diseases, lympho-proliferation and cancer. This is one of the most common forms of primary immunodeficiency disease, and severity varies from person to person.

Risk factors

This disorder is found in about one out of every 25,000 people, making it very common compared to other human immune defects. Both males and females are affected.

What are the signs and symptoms of pediatric common variable immunodeficiency (CVID)?

• Ear infections

• Sinusitis

• Pneumonia

• Chronic diarrhea and weight loss

• Gastrointestinal infections such as Giardia, Campylobacter

• Hepatitis

• Autoimmune problems involving the blood cells including:

  • ​​​​​​ Anemia (low red cells)

  • Thrombocytopenia (low platelets)

  • Neutropenia (low white cells)

How is pediatric common variable immunodeficiency (CVID) diagnosed?

Your child’s doctor may suspect CVID if he or she has a history of recurrent bacterial infections of the lungs, bronchi, ears or sinuses. The doctor will order screening tests that measure immunoglobulin levels in the blood and may also look for the number of B- and T-lymphocytes present.

The diagnostic criteria for CVID include the following:

• A child must be older than 4 years of age

• The child must have a low immunoglobulin G (IgG) AND either a low immunoglobulin A (IgA) or immunoglobulin M (IgM)

• The child must be unable to make specific antibodies to vaccines

What are the causes of pediatric common variable immunodeficiency (CVID)?

In the majority of cases, a genetic cause is not known. In less than 10 percent of patients, there may be a family history of CVID. In five - 10 percent of patients, a genetic cause may be identified, although this remains a research question.

How is pediatric common variable immunodeficiency (CVID) treated?

If your child is diagnosed with CVID, the doctor will likely administer immunoglobin replacement therapy, which usually relieves symptoms. Your child will need to get these treatments regularly throughout their life.

• IgG therapy - IgG therapy can be given through a catheter in your child’s vein (intravenous; IVIG) or subcutaneously (SCIG). The decision to use one or the other will depend on what’s best for your child and your family. 

• Medication - Your child’s doctor will also prescribe antibiotics to treat infections, and many times, longer courses may be required than for those patients without CVID.

• Biopsies - Sometimes, biopsies may be needed when nodules in the lung and liver appear. This may change treatments.

If lympho-proliferation and granulomas develop, other treatments may be needed to suppress the immune system further. These medicines include steroids and other chemotherapy medications.

All patients with CVID should be followed at the center. If patients with CVID have complications – including pulmonary, gastrointestinal, or liver problems – these require the cooperative interaction of your child’s immunologist with other subspecialties.