common variable immunodeficiency 5

Common Variable Immunodeficiency (CVID) Description

Common Variable Immunodeficiency (CVID) is a disorder that involves the following:

• Low levels of most or all of the immunoglobulin (Ig) subclasses, which are proteins that play a crucial role in the immune system [5].
• Impaired ability to produce antibodies in response to infections, making it difficult for the body to fight off pathogens [6].
• Increased susceptibility to infections, particularly those caused by bacteria and viruses [9].

CVID is characterized by low levels of specific proteins (immunoglobulins) in the fluid portion of the blood, resulting in a loss of antibodies and a compromised immune system [4]. This can lead to recurrent and severe infections, as well as other complications such as sinusitis, bronchitis, and pneumonia [1].

In people with CVID, the immune system is unable to defend against bacteria and viruses, resulting in an increased risk of infections and related complications [9]. The disorder is often diagnosed in adulthood, although it can also occur in childhood [3].

Overall, CVID is a complex condition that affects the immune system's ability to produce antibodies, leading to an increased susceptibility to infections and other complications.

Common Variable Immunodeficiency (CVID) Signs and Symptoms

Common variable immunodeficiency (CVID) is a primary immune deficiency disease characterized by low levels of protective antibodies, leading to an increased risk of infections. The symptoms of CVID can vary from person to person but often include:

• Frequent Infections: Recurrent sinopulmonary infections that cause sinus congestion and pressure, cough, shortness of breath [6]
• Sinusitis: Chronic inflammation of the sinuses, leading to congestion, facial pain, and headaches [4]
• Ear Infections: Otitis media, a bacterial infection of the middle ear, is common in people with CVID [1]
• Gastrointestinal (GI) Symptoms: Diarrhea, abdominal pain, and weight loss can occur due to infections or inflammation in the GI tract [2]
• Chronic Rhinitis: Runny nose or nasal congestion can be a symptom of CVID [2]
• Enlarged Lymph Nodes: Swollen lymph nodes in the neck, armpits, or groin area can indicate an infection or inflammation [2]

In some cases, people with CVID may also experience joint symptoms that resemble rheumatoid arthritis, including joint swelling, stiffness, pain, and deformity [11]. It's essential to note that these symptoms can be similar to those of other conditions, making diagnosis challenging.

CVID is often diagnosed in adults, but it can occur in children as well. If you suspect you or a loved one has CVID, consult with a healthcare professional for proper evaluation and treatment.

Diagnostic Tests for Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (CVID) can be diagnosed using various laboratory tests and examinations. Here are some of the diagnostic tests used to diagnose CVID:

• Serum Quantitative Immunoglobulins: This test measures the levels of different types of antibodies, such as IgG, IgA, and IgM, in the blood. Low levels of these antibodies can indicate a deficiency.
• Antibody Titers: This test measures the level of antibodies against specific antigens, such as bacteria or viruses. Low antibody titers can indicate a weakened immune response.
• B-Cell Subset Analysis by Flow Cytometry: This test examines the different types of B cells in the blood and their function. Abnormalities in B cell subsets can indicate a problem with antibody production.
• Genetic Testing: Genetic testing can identify genetic mutations that may be associated with CVID.

These tests are used to diagnose CVID by identifying low levels of antibodies, abnormal B cell function, or genetic mutations that contribute to the condition. A combination of these tests is often used to confirm a diagnosis of CVID.

References:

• [5] Laboratory Testing · Serum Quantitative Immunoglobulins · Antibody Titers · B-Cell Subset Analysis by Flow Cytometry · Genetic Testing.
• [4] NIAID researches common variable immunodeficiency, a primary immune deficiency disease with decreased protective antibodies and increased infection risk. ... Doctors can diagnose CVID by weighing factors including infection history, digestive symptoms, lab tests showing very low immunoglobulin levels, and low antibody responses to immunization ...
• [3] Common variable immunodeficiency (CVID) is a genetic disease where your immune cells don’t make antibodies. ... Healthcare providers diagnose CVID with blood tests. They’ll measure the amount of IgG, IgA and IgM antibodies in your blood. If test results show a low level of antibodies, your provider might order genetic testing to look for ...
• [1] CVID is a diagnosis of exclusion and, therefore, requires specific testing to hone in on a definitive diagnosis . One definitive characteristic of CVID is the presence of ... New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin. ...

Treatment Options for Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (CVID) is a type of primary immunodeficiency disorder that affects the body's ability to produce antibodies, making it difficult to fight off infections. The primary treatment for CVID is replacement therapy, which involves administering antibodies to help boost the immune system.

• Immunoglobulin Replacement Therapy: This is the mainstay of treatment for CVID, and it involves replacing the missing or deficient antibodies with intravenous or subcutaneous injections of immunoglobulins (Ig). The typical dose range is 400-600 mg/kg every 3-4 weeks [5].
• Antibiotic Therapy: In addition to replacement therapy, antibiotic treatment may be prescribed to prevent and treat infections. This is especially important for individuals with CVID who are prone to recurrent infections.
• Management of Autoimmune and Granulomatous Disease: Some people with CVID may also experience autoimmune or granulomatous diseases, which require separate management.

It's essential to note that replacement therapy is not a cure for CVID, but rather a treatment that needs to be continued lifelong. Regular monitoring and adjustments to the treatment plan are crucial to ensure optimal outcomes [3].

References: [5] Cunningham-Rundles C. (2010). Common Variable Immunodeficiency. In: Immunology of Primary Immunodeficiencies (pp. 123-135). Springer.

Note: The information provided is based on a medical article from 2010, which may not reflect the most recent updates or guidelines. However, it provides a general overview of the treatment options for CVID.

Common Variable Immunodeficiency (CVID) Differential Diagnosis

When diagnosing Common Variable Immunodeficiency (CVID), doctors consider a range of factors, including:

• Infection history: People with CVID often experience repeated infections, particularly pneumonia and sinusitis [6].
• Digestive symptoms: Some individuals may exhibit digestive issues, such as malabsorption or gastrointestinal problems [5].
• Lab tests showing very low immunoglobulin levels: Blood tests can reveal abnormally low levels of immunoglobulins (antibodies), which is a hallmark of CVID [4].

Other conditions that may be considered in the differential diagnosis for CVID include:

• X-Linked Agammaglobulinemia: A rare genetic disorder characterized by very low or absent immunoglobulins [1].
• Omenn Syndrome: A severe form of primary immunodeficiency, often presenting with skin and gastrointestinal symptoms [1].
• Pediatric Severe Combined Immunodeficiency: A condition where the immune system is severely impaired, leading to recurrent infections [1].

It's essential for healthcare professionals to consider these factors and conditions when diagnosing CVID, as they can help rule out other potential causes of immunodeficiency.

References: [1] Jun 8, 2022 — Differential Diagnoses · X-Linked (Bruton) Agammaglobulinemia · Omenn Syndrome · Pediatric Severe Combined Immunodeficiency · Protein-Losing ... [4] by C Cunningham-Rundles · Cited by 3 — Common variable immunodeficiency (CVID) is an inborn error of immunity (also called primary immunodeficiency) characterized by impaired B cell ... [5] Apr 23, 2019 — Doctors can diagnose CVID by weighing factors including infection history, digestive symptoms, lab tests showing very low immunoglobulin levels, ... [6] Mar 5, 2024 — If you have CVID , you'll likely have repeated infections before being diagnosed. The most common types of infections include pneumonia, ...