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Common Variable Immunodeficiency (CVID)

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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.

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 

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 5-10 percent of patients, a genetic cause may be identified, although this remains a research question. 

The Primary Immunodeficiency Program at Children’s Medical Center has been recognized as a Center of Excellence by the Jeffrey Modell Foundation for the diagnosis and treatment of patients with primary immunodeficiency including CVID.

Symptoms

Symptoms

CVID can be diagnosed anytime from childhood through adulthood.

The most common symptoms are recurrent infections of the ears, sinuses, lungs, liver, and gastrointestinal tract including:

  • Ear infections
  • Sinusitis
  • Pneumonia
  • Chronic diarrhea and weight loss
  • Hepatitis
  • Autoimmune problems involving the blood cells including anemia ( low red cells), thrombocytopenia ( low platelets) , neutropenia ( low white cells)
  • Gastrointestinal infections such as Giardia, Campylobacter 

If lung infections are severe and occur repeatedly, a chronic condition of the bronchi (breathing tubes) can develop, causing widening and scarring of these structures called bronchiectasis.

Infections occurring in children with CVID are often due to common bacteria like hemophilus influenzae, pneumococci and staphylococci.

Children with CVID may develop a process of lympho-proliferation. This means that lymphocytes, the important cells of the immune system, start dividing and accumulating in the lymphoid organs such as the lymph nodes, spleen, gut, and liver.  This causes the lymph nodes in the neck, chest, or abdomen to enlarge, and the spleen and liver can grow. The intestinal walls also become swollen and cannot absorb nutrients well.

Sometimes, these collections of growing lymphocytes form balls of cells called granulomas. These cause nodules in the lungs, liver, spleen and gut

Rarely, children with CVID can develop cancers. This is seen more commonly in adults.

Tests and Diagnosis

Tests and Diagnosis

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. Other tests may include:

  • Immunization with killed vaccines to determine antibody function, a key test that helps the doctor decide if your child will benefit from immunoglobulin replacement therapy.
  • Radiology studies of the neck, chest, and abdomen.
  • Lung function studies.
Treatments

Treatments

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 his or her life.

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. 

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.

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.

FAQs

FAQs

How common is common variable immunodeficiency?

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.

Why is CVID called “variable?”

Variable is included in the disorder’s name because its clinical course varies from patient to patient.

Does everyone with CVID need immunoglobin replacement therapy?

Yes, this is the most effective form of treatment.

Are there any conditions associated with CVID?

Sometimes people with CVID can also develop autoimmune cytopenias including thrombocytopenia (most common), anemia or neutropenia. Lupus in not commonly seen in patients with CVID.

Where can I find a support group?

We will provide you with resources to help both you and your child. The Resources link on this webpage is also a good source for more information about common variable immunodeficiency and support groups. You can also visit the Jeffrey Modell foundation (www.info4pi.org/).

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