Common Variable Immunodeficiency (CVID)

1 What is Common Variable Immunodeficiency (CVID)?

Common variable immunodeficiency (CVID) is a rare immune disorder which impairs your immune system and increases susceptibility to infection.

If you have CVID, the level of antibodies in your body is low which makes you prone to recurrent infections of ear, sinuses and respiratory systems. You are also likely to develop digestive disorders, blood disorders and cancer if you have CVID.

The cause of CVID is unknown. Symptoms and severity of the disease vary widely. Typically, the signs and symptoms appear in adulthood, however, some people may experience them during childhood or adolescence. 

2 Symptoms

Common Variable Immunodeficiency (CVID) can present in a various amount of symptoms, ranging from infections to skin conditions.

People with CVID experience:

  • Recurrent respiratory tract infections such as pneumonia, sinusitis. Recurrent infections may lead to permanently injured bronchi and cause bronchiectasis
  • Autoimmune diseases: Autoimmune diseases occur when your immune system mistakenly attacks your own healthy cells. Approximately one fifth of people with CVID are found to have autoimmune disorders, such as rheumatoid arthritis, vitiligo, hemolytic anemia, thrombocytopenia, neutropenia, and gastrointestinal diseases.
  • Malignancy: CVID increases risk of certain malignancies such as, lymphomas of B-cell.
  • Persistent giardiasis (diarrhea caused my microorganism called Giardia lamblia) leading to malabsorption.
  • Children with CVID show growth retardation.

Other signs and symptoms that are seen in the skin of people with CVID include:

  • Hair loss, either patches of hair or all of the hair falls out
  • Rashes
  • Ulcerative nodules
  • Inflammation of blood vessels in skin (cutaneous vasculitis)

Most people with CVID have lesions on the face, legs and hands, and contain no microorganism. The lesions disappear after treating the cause.

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3 Causes

The exact cause of common variable immunodeficiency (CVID) is not known. It is thought to result from a combination of genetic factors and environmental factors. 

4 Making a Diagnosis

Making a diagnosis of common variable immunodeficiency (CVID) is done by performing various tests and procedures that include:

Laboratory tests

These tests analyze your blood sample to determine the levels of various antibodies (infection-fighting proteins produced by cells in your body) and other types of white blood cells.

If you have CVID, your blood analysis will reveal:

  • Decreased level of serum antibodies such as, IgA and IgG
  • In rare cases, decreased level of serum IgM
  • Reduced number of T and B lymphocytes in blood

Imaging studies

High-resolution CT scan of the chest can be used to check abnormalities in lungs.

Histology

Microscopic studies of cells may show abnormal cell multiplication in your lymph nodes.

Procedures

Biopsy

A sample of tissue from lymph nodes may be removed (biopsy) to check whether the enlarged lymph nodes are result of certain conditions, such as infection or malignancy.

Bronchoscopy or endoscopy

It is performed to check if any lesions or infections in bronchi are causing the symptoms.

5 Treatment

Replacing antibodies called Immunoglobulin (Ig) to prevent recurrent infections is the primary treatment for common variable immunodeficiency (CVID).

Ig can be given directly into your vein (intravenously) or under your skin (subcutaneously).

The usual dose is 400-600 mg/kg every 2-4 weeks. 

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