common variable immunodeficiency 6

Common Variable Immunodeficiency (CVID) Description

Common variable immunodeficiency (CVID) is a disorder that impairs the immune system, making people highly susceptible to infection from foreign invaders such as bacteria or viruses. This condition is characterized by low levels of specific proteins (immunoglobulins) in the fluid portion of the blood, resulting in a loss of antibodies and an increased risk of infections.

Key Features:

• Low levels of serum antibodies
• Increased susceptibility to infection
• Frequent infections in the ears, sinuses, nose, and lungs
• Reduced antibody responses

According to [6], CVID is characterized by low levels of most or all of the immunoglobulin (Ig) classes. This lack of B lymphocytes or plasma cells that are capable of producing antibodies leads to a compromised immune system.

Prevalence:

CVID affects about 1 in every 25,000 people [14]. The causes of CVID are varied and can be attributed to more than a dozen genes associated with the disorder, accounting for approximately 10% of people with the condition.

Common Variable Immunodeficiency (CVID) Signs and Symptoms

Patients with CVID experience a range of symptoms, primarily affecting the respiratory system, ears, and gastrointestinal tract.

• Recurrent Sinopulmonary Infections: Patients often have repeated infections that cause sinus congestion and pressure, cough, shortness of breath, and other respiratory problems.
• Gastrointestinal Problems: Gastrointestinal issues such as chronic diarrhea, weight loss, nausea, vomiting, and abdominal pain can also be present in some forms of CVID.

These symptoms are a result of the body's inability to produce sufficient antibodies to fight off infections, making patients more susceptible to illnesses. [6]

Diagnostic Tests for Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (CVID) can be diagnosed through various tests that assess the immune system's function and antibody production. Here are some diagnostic tests used to diagnose CVID:

• Blood tests: Blood tests measure the levels of different types of antibodies, such as IgG, IgA, and IgM, in the blood [1]. Low levels of these antibodies can indicate a deficiency in the immune system.
• Immunoglobulin (antibody) levels: Doctors measure immunoglobulin levels to determine if there is a lack of functional antibody production [5].
• Lab tests showing very low immunoglobulin levels: Lab tests that show very low levels of immunoglobulins can indicate CVID [9].
• Low antibody responses to immunization: Measuring the body's response to vaccines can help diagnose CVID by determining how well the body produces antibodies in response to vaccination [8].

These diagnostic tests are used to determine if an individual has a deficiency in their immune system, which is characteristic of CVID. A diagnosis of CVID is usually confirmed by abnormal blood test results and medical history [9].

Treatment for Common Variable Immunodeficiency (CVID)

The primary treatment for CVID involves replacing antibodies, which can be achieved through intravenous or subcutaneous routes of Ig (immunoglobulin) therapy. This is typically done in doses of 400 to 600 mg/kg every 3-4 weeks [5]. In addition to immunoglobulin replacement, preventative antibiotics are also used to manage infections and prevent complications.

The goal of treatment for CVID is to reduce the frequency and severity of infections, as well as to improve overall quality of life. This can be achieved through a combination of immunoglobulin replacement therapy and antibiotic prophylaxis [6].

Some key points to consider when it comes to treating CVID include:

• Immunoglobulin Replacement: This is the cornerstone of treatment for CVID, and involves replacing antibodies that are deficient in individuals with this condition.
• Preventative Antibiotics: These are used to prevent infections and reduce the risk of complications.
• Regular Monitoring: Regular monitoring by a healthcare provider is essential to ensure that treatment is effective and to make any necessary adjustments.

Overall, the goal of treatment for CVID is to improve quality of life and reduce the risk of complications. With proper management, individuals with this condition can lead active and healthy lives [8].

References:

[5] Cunningham-Rundles C. (2010). Common Variable Immunodeficiency: A Review. Journal of Clinical Immunology, 30(1), 1-11.

[6] Treatment for CVID involves immunoglobulin replacement, preventative antibiotics, and if indicated management of autoimmune and granulomatous disease. Keep pace with the latest research and guidelines to ensure optimal care for your patients.

[8] Mar 5, 2024 — Treatments for CVID focus on decreasing how often you get infections and how bad they are. Treatments may include immunoglobulin and antibiotic therapy.

Differential Diagnosis of Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) can be challenging to diagnose due to its heterogeneous presentation and the presence of various differential diagnoses. The following conditions should be considered in the differential diagnosis of CVID:

• Other causes of hypogammaglobulinemia: These include loss of gammaglobulins via the intestine or urine, which can lead to similar clinical presentations as CVID.
• Monoclonal protein levels for monoclonal gammopathy: Elevated monoclonal protein levels can be indicative of monoclonal gammopathy, a condition that can mimic CVID in its presentation.

Key Points:

• Differential diagnosis is crucial in the evaluation of CVID to rule out other conditions that may present similarly.
• Other causes of hypogammaglobulinemia and monoclonal gammopathy should be considered in the differential diagnosis of CVID.
• A thorough clinical evaluation, including laboratory tests, is necessary to establish a definitive diagnosis.

References:

• [6] Mar 5, 2024 — Common variable immunodeficiency, also called CVID, is an immune system disorder that causes low levels of the proteins in the body that help fight infections.
• [8] Differential diagnosis include other causes of hypogammaglobulinemia including loss of gammaglobulins via the intestine or urine ...
• [11] Other tests that can rule out differential diagnoses include monoclonal protein levels for monoclonal ...