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common variable immunodeficiency 10

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Description

Common Variable Immunodeficiency (CVID) Description

Common Variable Immunodeficiency (CVID) is a primary immune deficiency disease characterized by low levels of protective antibodies and an increased susceptibility to infection [1]. It is also known as antibody deficiency, which leaves the immune system unable to defend against bacteria and viruses [9].

Key Features:

  • Low levels of serum antibodies, leading to increased susceptibility to infection [2]
  • Relatively frequent form of primary immunodeficiency (PID) disorders [2]
  • Characterized by low levels of specific proteins (immunoglobulins) in the fluid portion of the blood [4]
  • Results in a loss of antibodies and a compromised immune system [9]

Symptoms:

  • Increased susceptibility to infection, particularly respiratory tract infections [1]
  • Recurrent or chronic infections, such as pneumonia, sinusitis, or bronchitis [6]
  • Frequent or severe infections that may require hospitalization [7]

Note: The description of CVID is based on the provided context and search results.

Additional Characteristics

  • Common Variable Immunodeficiency (CVID) is a primary immune deficiency disease characterized by low levels of protective antibodies and an increased susceptibility to infection
  • Low levels of serum antibodies, leading to increased susceptibility to infection
  • Characterized by low levels of specific proteins (immunoglobulins) in the fluid portion of the blood
  • Increased susceptibility to infection, particularly respiratory tract infections
  • Recurrent or chronic infections, such as pneumonia, sinusitis, or bronchitis

Signs and Symptoms

Common Variable Immunodeficiency (CVID) Signs and Symptoms

People with CVID may experience a range of signs and symptoms, which can vary from person to person. Some of the most common symptoms include:

  • Recurring sinus and lung infections: Pneumonia, bronchitis, and other respiratory tract infections are common in people with CVID.
  • Joint symptoms: About 1%–10% of people with CVID may experience joint swelling, stiffness, pain, and deformity, which can mimic rheumatoid arthritis.
  • Chronic cough: A persistent cough that brings up mucus or blood is a common symptom of CVID.
  • Difficulty breathing: People with frequent lung infections can develop bronchiectasis, making it harder to breathe.
  • Sinus congestion and pressure: Sinusitis and other sinus problems are common in people with CVID.

These symptoms can be similar to those experienced by people with other conditions, which can make diagnosis challenging. However, if you or a loved one is experiencing recurring infections or other symptoms that persist over time, it's essential to consult a healthcare professional for proper evaluation and treatment.

References:

  • [10] - About 1%–10% of people with common variable immunodeficiency will have joint symptoms that, at first glance, appear like rheumatoid arthritis. These exact symptoms can include joint swelling, stiffness, pain, and deformity.
  • [13] - Recurring sinus and lung infections, particularly pneumonia, are common.People may develop a chronic cough, cough up blood, or have difficulty breathing.
  • [14] - People with the same type of CVID may have varying signs and symptoms.

Additional Symptoms

  • Recurring sinus and lung infections
  • Joint swelling, stiffness, pain, and deformity
  • Chronic cough that brings up mucus or blood
  • Difficulty breathing due to bronchiectasis
  • Sinus congestion and pressure from sinusitis

Diagnostic Tests

Diagnostic Tests for Common Variable Immunodeficiency (CVID)

Diagnosing CVID involves a combination of medical history, physical examination, and laboratory tests to rule out other immune conditions. The diagnostic process typically includes:

  • Blood tests: To assess immunoglobulin (antibody) levels, including IgG, IgA, and IgM antibodies.
  • Genetic testing: May be ordered if blood test results show a low level of antibodies to look for genetic mutations that can cause CVID.

These diagnostic tests help healthcare providers determine the presence of CVID by identifying:

  • Low levels of immunoglobulins (antibodies) in the blood
  • Reduced antibody responses to vaccines and infections
  • Presence of recurrent sinopulmonary infections, autoimmune disorders, or granulomatous diseases

Key Points:

  • CVID is a diagnosis of exclusion, requiring specific testing to rule out other immune conditions.
  • Blood tests are essential for diagnosing CVID, as they help assess immunoglobulin levels and identify potential genetic mutations.
  • Genetic testing may be ordered if blood test results show low antibody levels.

References:

[10] 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 [10][11]

Treatment

Treatment Options for Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (CVID) is a type of primary immunodeficiency disorder that affects the immune system's ability to produce antibodies. The cornerstone of therapy for CVID is immune globulin replacement, which has dramatically altered the clinical course of the disease by reducing the burden of infections [2].

Immunoglobulin Replacement Therapy

The mainstay of treatment for CVID is Ig replacement therapy, which involves administering immunoglobulins (antibodies) to help boost the immune system's ability to fight off infections [4]. This type of therapy can be administered intravenously or subcutaneously, and the frequency of administration may vary depending on individual needs [8].

Antibiotic Therapy

In addition to immunoglobulin replacement therapy, antibiotic therapy is also a crucial component of CVID treatment. Prophylactic antibiotics are often prescribed to prevent infections and reduce their severity [7]. The goal of this treatment approach is to keep the individual as healthy as possible by reducing the frequency and severity of infections.

Other Treatment Options

While immunoglobulin replacement therapy and antibiotic therapy are the primary treatments for CVID, other options may also be considered on a case-by-case basis. These may include antiviral therapy, management of autoimmune and granulomatous disease, and other supportive measures to help manage symptoms and improve quality of life [6].

Important Considerations

It's essential to note that replacement therapy is not a cure for CVID – individuals will need to continue receiving treatment for the rest of their lives [3]. Regular monitoring and follow-up care with a healthcare provider are crucial to ensure optimal management of the condition.

References: [1] Not applicable (no relevant information available) [2] Sep 15, 2022 [3] Jun 8, 2022 [4] Jun 8, 2022 [5] Not applicable (no relevant information available) [6] Treatment for CVID involves immunoglobulin replacement, preventative antibiotics, and if indicated management of autoimmune and granulomatous disease. Keep pace by MAP Ruschel · 2023 · Cited by 5 — Describe the treatment and management options available for common variable immunodeficiency disease. [7] by MAP Ruschel · 2023 · Cited by 5 — Describe the treatment and management options available for common variable immunodeficiency disease. Prophylactic treatment may include antiviral therapy. 3. [8] It may be injected into a vein (intravenously) once a month or under the skin (subcutaneously) once a week or once a month. Antibiotics are promptly given to ... [9] 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 ...

Recommended Medications

  • Antibiotic therapy
  • Ig replacement therapy
  • Antiviral therapy

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnoses for Common Variable Immunodeficiency (CVID)

Common variable immunodeficiency (CVID) is a primary immune deficiency disorder characterized by impaired B-cell differentiation and function, leading to recurrent infections and hypogammaglobulinemia. However, there are several other conditions that can present with similar symptoms, making differential diagnosis crucial for accurate diagnosis.

Other Causes of Hypogammaglobulinemia:

  • Loss of gammaglobulins via the intestine or urine (e.g., gastrointestinal disorders, kidney disease)
  • Secondary hypogammaglobulinemia due to chronic infections, autoimmune diseases, or malignancies
  • X-linked agammaglobulinemia (Bruton's disease)
  • Omenn syndrome
  • Pediatric severe combined immunodeficiency

Conditions to Consider in the Differential Diagnosis:

  • Recurrent infections and alopecia should prompt consideration of CVID in the differential diagnosis [10]
  • Other primary immune deficiency disorders, such as X-linked agammaglobulinemia (Bruton's disease), Omenn syndrome, and pediatric severe combined immunodeficiency, can present with similar symptoms
  • Secondary causes of hypogammaglobulinemia, such as chronic infections or autoimmune diseases, should also be considered

References:

[1] MAP Ruschel. The basis for differential diagnosis is on the main laboratory characteristic of CVID: hypogammaglobulinemia. [3] JS Tam. Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent bacterial infections and impaired B-cell differentiation leading... [9] Differential diagnosis include other causes of hypogammaglobulinemia including loss of gammaglobulins via the intestine or urine...

Additional Differential Diagnoses

  • Loss of gammaglobulins via the intestine or urine
  • Pediatric severe combined immunodeficiency
  • Secondary hypogammaglobulinemia due to chronic infections, autoimmune diseases, or malignancies
  • Omenn syndrome
  • X-linked agammaglobulinemia

Additional Information

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