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· Common variable immunodeficiency (CVID), the most prevalent humoral immunodeficiency, is characterized by hypogammaglobulinemia, impaired vaccine responses, and recurrent sinopulmonary infections.1 Although known risk factors for more severe COVID-19 infection include older age, hypertension (HTN), obesity, and cardiovascular disease,2 the …
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How do you test for common variable immune deficiency?
The diagnosis of CVID is primarily established by testing for low blood (serum) IgG immunoglobulin concentrations ranging from severely reduced (<100 mg/dL) to just below adult normal range (500-1200 mg/dL). In addition, laboratory testing may reveal normal or, in some cases, reduced numbers of circulating B cells.
What is common variable immunodeficiency CVID?
Common variable immunodeficiency (CVID) is a primary immune deficiency disease characterized by low levels of protective antibodies and an increased risk of infections. Although the disease usually is diagnosed in adults, it also can occur in children.
What are the common signs and symptoms of common variable immunodeficiency CVID )?
What are the symptoms of common variable immunodeficiency (CVID)?Breathing problems.Chronic cough.Diarrhea that causes weight loss.Ear infections.Frequent sinus infections.Recurring lung infections, including pneumonia.
When do you suspect common variable immunodeficiency?
Diagnosis of Common Variable Immune Deficiency CVID should be suspected in children or adults who have a history of recurrent bacterial infections involving ears, sinuses, bronchi and lungs. The characteristic laboratory features include low levels of serum immunoglobulins, including IgG, often IgA and sometimes IgM.
What is considered a low IgG level?
Serum IgG levels reduced by more than 2 SDs below the mean, accompanied by deficiencies in IgA and/or IgM, suggest common variable immunodeficiency. Usually the IgG level is less than 400 mg/dL; 70% of patients will have very low or absent IgA content.
What causes a low IgM level?
Other causes of decreased levels of serum IgM (i.e., secondary IgM deficiency) are episodes of infection, thymic hypoplasia, celiac disease, autoimmune disease, and certain adult malignancies; and other PIDs (Wiskott-Aldrich syndrome, DOCK8 deficiency, ataxia-telangiectasia, CVID, and XLA, in combination with IgG and ...
What is common immune deficiency?
Common variable immune deficiency (CVID) is a disorder that impairs the immune system. People with CVID are highly susceptible to infection from foreign invaders such as bacteria, or more rarely, viruses and often develop recurrent infections, particularly in the lungs , sinuses , and ears .
What are the symptoms of immunoglobulin deficiencies?
What are the symptoms of an IgG deficiency?Sinus infections and other respiratory infections.Gastrointestinal infections.Ear infections.Pneumonia.Bronchitis.Infections that result in a sore throat.Rarely, severe and life-threatening infections.
What causes low IgG levels?
Secondary IgG deficiency may be caused by: Aging. Malnutrition. Chemotherapy medicines and long-term corticosteroids.
What does low IgG IgA and IGM mean?
Low levels of immunoglobulins mean your immune system isn't working as well as it should. This can be caused by: Medicines that weaken your immune system, such as steroids. Diabetes complications. Kidney disease or kidney failure.
What is Hypo gamma globulin anemia?
Hypogammaglobulinemia is a problem with the immune system that prevents it from making enough antibodies called immunoglobulins. Antibodies are proteins that help your body recognize and fight off foreign invaders like bacteria, viruses, and fungi. Without enough antibodies, you're more likely to get infections.
Why is it called common variable immunodeficiency?
The reason it was termed “common” is related to the original WHO decision to lump several disorders under the label “common variable immunodeficiency" since considering the proportion of all immunodeficiency disorders that fell under that heading, it was the most common, yet variable (the WHO chose the name, not IDF).
Symptoms
The clinical course and symptoms of CVID vary widely from mild to severe. The immunoglobulins affected also vary. For example, some patients have a deficiency in all three major types of immumoglobulins: immunoglobulin G (IgG), immunoglobulin A (IgA), and immunoglobulin M (IgM) while others have a shortage of just IgG and IgA. The diagnosis is made...
Individuals with CVID also have an increased susceptibility to certain bacterial gastrointestinal infections (e.g., Campylobacter, etc.) that may cause symptoms similar to those associated with giardiasis.
Due to abnormalities in the maturation of B cells, and dysregulation of the immune system, some individuals with CVID may have abnormal accumulations of lymphocytes in lymphoid tissues such as lymph nodes (lymphadenopathy) or spleen (splenomegaly). In some cases, abnormal growth of small nodules of lymphoid tissue in the gastrointestinal tract (nodular lymphoid hyperplasia) …
It is not fully understood why CVID patients are at risk for autoimmune disorders. CVID suppresses the immune response, whereas autoimmunity involves an overactive immune system that attack the bodys healthy tissues and organs. This phenomenon suggests that more complex defects in the immune system, beyond qualitative and quantitative defects in antibodies product…
The cause of CVID is unknown for most patients but a genetic cause has been identified in about 10%. Autosomal dominant and autosomal recessive inheritance has been reported in a few families with CVID. More commonly, sporadic cases, with no apparent history of the disorder in their family, may still be caused by either rare autosomal defects or complex interactions of envi…
Mutations in at least five genes have been associated with CVID. Approximately 10 percent of affected individuals have mutations in the TNFRSF13B gene but as the same gene can be found in normal relatives and blood bank normal donors, it is not considered a direct cause of CVID. Other genes that have been associated with a very small percentage of cases include: CD19, CD81, IC…
CVID equally affects males and females. The prevalence of CVID is approximately 1 in 30,000 people. The diagnosis of CVID is not made in children under the age of 4, because until that time, it may be confused with other genetic defects that must be excluded. In addition, it can be confused with physiologic immaturity. However, most patients have symptoms later and are no…
Symptoms of the following disorders may be similar to those of Common Variable Immunodeficiency. Comparisons may be useful for a differential diagnosis: In most patients, Common Variable Immune Deficiency is diagnosed based upon a thorough clinical evaluation, identification of characteristic symptoms and physical findings, a detailed patient and family his…
Agammaglobulinemia is a group of inherited immune deficiencies characterized by a low concentration of antibodies in the blood due to the lack of particular lymphocytes in the blood and lymph. The types of agammaglobulinemia are: X-linked agammaglobulinemia (XLA), the much rarer X-linked agammaglobulinemia with growth hormone deficiency (about 10 cases reported), …
Hyper-IgM Syndrome (HIGM) is a rare primary immunodeficiency disorder that is usually inherited as an X-linked recessive condition. People with this disorder have low levels of IgG, IgA and IgE antibodies. Levels of IgM antibodies may be high or in the normal range. Symptoms and physical findings usually become apparent in the first or second year of life. HIGM is characterized by rec…
Treatment The treatment of Common Variable Immune Deficiency requires the coordinated efforts of a team of specialists who may need to systematically and comprehensively plan an affected individuals treatment. Such specialists may include physicians who diagnose and treat disorders of the blood (hematologists), the digestive tract (gastroenterologists), and/or the lung…
Some researchers have recommended that when a patient is diagnosed with an autoimmune disease, the possibility of an underlying CVID should be evaluated before the administration of immunosuppressive drugs for the autoimmune disease.
Affected individuals with severely low levels of circulating platelets may be cautioned to avoid the use of aspirin, since this medication may interfere with the ability of platelets to assist in the blood-clotting process. In addition, as is the case with individuals affected by many other primary immunodeficiency disorders, individuals with CVID should not receive live virus vaccines since t…
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