Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrognosis
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Prognosis
Common variable immunodeficiency(CVID) is an immune system disorder that causes low antibodies, which reduces the body’s ability to fight off infections. As a result, people with CVID are highly susceptible to frequent and sometimes severe infections, as well as complications from these infections.
This article provides an overview of CVID, including symptoms, causes, diagnosis, and treatment.
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The hallmarksymptom of CVIDis recurrent and potentially severe viral or bacterial infections.The precise symptoms will differ based on what type of infection the person has.
For example, different types of infections (e.g., upper respiratory tract infections,pneumonia, gastrointestinal infections, ear infections) will all present with very different symptoms. However, it is the frequent recurrence of these infections that indicates a warning sign for CVID, rather than any exact symptoms of the infection itself.
Other than acute infections, there may be complications such as:
Prevalence of CVIDCVID is the most commonprimary immunodeficiency, but this does not mean it is a common disorder. Estimates vary, but it is thought that CVID occurs in anywhere from one in 10,000 to one in 50,000 people.
Prevalence of CVID
CVID is the most commonprimary immunodeficiency, but this does not mean it is a common disorder. Estimates vary, but it is thought that CVID occurs in anywhere from one in 10,000 to one in 50,000 people.
B-cellsare a type of white blood cell that producesantibodies. Antibodies are the Y-shaped proteins that help us fend off infections. CVID causes a deficiency in these antibodies.
Over 20 different genetic mutations have been identified so far as either causing CVID or increasing a person’s risk of developing CVID.The most common are mutations in the TNFRSF13B gene (approximately 8% of CVID cases) and the NFKB1 gene.
Most of these genetic mutations occur sporadically, but some may be passed down through families. This can occur through both dominant and recessive inheritance.
However, it is important to note that identified genetic mutations only explain 10% to 20% of CVID cases.This leads researchers to believe that environmental factors play a role in causing CVID, although we do not know at this time what these environmental factors might be. There are also likely many other genes that cause CVID that have yet to be identified.
After gathering a health history of recurrent infections,CVID can be diagnosedthrough a series of blood tests.
These include tests such as:
To be diagnosed with CVID, a person must have low immunoglobulins (antibodies). This includes low IgG and either IgM or IgA.It also requires a poor response to vaccines and the absence of any other disorder that causes an immunodeficiency.
Diagnostic Delay
Getting diagnosed with CVID can be challenging in multiple ways:
As a result, diagnosis can be delayed from four to nine years after symptoms begin.
This is a significant issue, because the longer CVID goes untreated, the more complications can form, which can impact life expectancy. Therefore, it’s essential to receive a timely, accurate diagnosis if you have symptoms of CVID.
The mainstaytreatment for CVIDis immunoglobulin (antibody) replacement therapy.This uses the blood donations from healthy individuals and essentially restores the antibodies in a person’s body to a normal level so they can better fight off infections.
Immunoglobulin replacement therapy can be given either through intravenous (IV) infusions or subcutaneous injections.The exact frequency and dosage should be determined individually, based on the person’s antibody level response.Treatment is lifelong.
Another aspect of CVID treatment is treating any active infections. Even with immunoglobulin replacement therapy, a person with CVID may experience more infections than the average person. This treatment will depend on what kind of specific infection the person has, but may include antibiotics, antivirals, over-the-counter (OTC) therapies, and more.
The good news is that life expectancy for those who have CVID has significantly improved in the last 30 years, from just 12 years post-diagnosis to over 50 years.This is thanks, in large part, to the pioneering of immunoglobulin replacement therapy as a CVID treatment.
However, some factors may reduce a person’s life expectancy. These include:
As a result, it’s important to receive a prompt diagnosis of CVID to prevent these complications.
The majority of people receiving immunoglobulin replacement therapy find they have fewer infections and a higher quality of life.
Summary
A Word From Verywell
It’s possible to live a full and active life with CVID, but it will take some adjustments. These include regular treatment with immunoglobulin replacement therapy, as well as lifestyle modifications to prevent infections. Finding a healthcare provider to help with either diagnosing or managing your CVID is an important first step in living with this condition.
13 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Institute of Allergy and Infectious Diseases.Common variable immunodeficiency (CVID).Saikia B, Gupta S.Common variable immunodeficiency.Indian J Pediatr. 2016;83(4):338-344. doi:10.1007/s12098-016-2038-xAmerican Academy of Allergy Asthma & Immunology.Common variable immunodeficiency: symptoms and diagnosis.Salzer U, Warnatz K, Peter HH.Common variable immunodeficiency - an update.Arthritis Research & Therapy. 2012;14(5):223. doi:10.1186/ar4032Ameratunga R, Longhurst H, Steele R, et al.Common variable immunodeficiency disorders, T-cell responses to SARS-CoV-2 vaccines, and the risk of chronic COVID-19.J Allergy Clin Immunol Pract. 2021;9(10):3575–3583. doi:10.1016/j.jaip.2021.06.019Genetic and Rare Diseases Information Center.Common variable immunodeficiency.MedlinePlus.Common variable immunodeficiency.National Organization for Rare Disorders.Common variable immunodeficiency.de Valles-Ibáñez G, Esteve-Solé A, Piquer M, et al.Evaluating the genetics of common variable immunodeficiency: monogenetic model and beyond.Front Immunol. 2018;9:636. doi:10.3389/fimmu.2018.00636Sperlich JM, Grimbacher B, Workman S, et al.Respiratory infections and antibiotic usage in common variable immunodeficiency.J Allergy Clin Immunol Pract.. 2018;6(1):159-168.e3. doi:10.1016/j.jaip.2017.05.024Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R.New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin.Clin Exp Immunol. 2013;174(2):203-211. doi:10.1111/cei.12178Ameratunga R, Woon S-T.Perspective: evolving concepts in the diagnosis and understanding of common variable immunodeficiency disorders(CVID).Clinic Rev Allerg Immunol. 2020;59(1):109-121. doi:10.1007/s12016-019-08765-6Cleveland Clinic.Common variable immunodeficiency (CVID).
13 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.National Institute of Allergy and Infectious Diseases.Common variable immunodeficiency (CVID).Saikia B, Gupta S.Common variable immunodeficiency.Indian J Pediatr. 2016;83(4):338-344. doi:10.1007/s12098-016-2038-xAmerican Academy of Allergy Asthma & Immunology.Common variable immunodeficiency: symptoms and diagnosis.Salzer U, Warnatz K, Peter HH.Common variable immunodeficiency - an update.Arthritis Research & Therapy. 2012;14(5):223. doi:10.1186/ar4032Ameratunga R, Longhurst H, Steele R, et al.Common variable immunodeficiency disorders, T-cell responses to SARS-CoV-2 vaccines, and the risk of chronic COVID-19.J Allergy Clin Immunol Pract. 2021;9(10):3575–3583. doi:10.1016/j.jaip.2021.06.019Genetic and Rare Diseases Information Center.Common variable immunodeficiency.MedlinePlus.Common variable immunodeficiency.National Organization for Rare Disorders.Common variable immunodeficiency.de Valles-Ibáñez G, Esteve-Solé A, Piquer M, et al.Evaluating the genetics of common variable immunodeficiency: monogenetic model and beyond.Front Immunol. 2018;9:636. doi:10.3389/fimmu.2018.00636Sperlich JM, Grimbacher B, Workman S, et al.Respiratory infections and antibiotic usage in common variable immunodeficiency.J Allergy Clin Immunol Pract.. 2018;6(1):159-168.e3. doi:10.1016/j.jaip.2017.05.024Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R.New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin.Clin Exp Immunol. 2013;174(2):203-211. doi:10.1111/cei.12178Ameratunga R, Woon S-T.Perspective: evolving concepts in the diagnosis and understanding of common variable immunodeficiency disorders(CVID).Clinic Rev Allerg Immunol. 2020;59(1):109-121. doi:10.1007/s12016-019-08765-6Cleveland Clinic.Common variable immunodeficiency (CVID).
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
National Institute of Allergy and Infectious Diseases.Common variable immunodeficiency (CVID).Saikia B, Gupta S.Common variable immunodeficiency.Indian J Pediatr. 2016;83(4):338-344. doi:10.1007/s12098-016-2038-xAmerican Academy of Allergy Asthma & Immunology.Common variable immunodeficiency: symptoms and diagnosis.Salzer U, Warnatz K, Peter HH.Common variable immunodeficiency - an update.Arthritis Research & Therapy. 2012;14(5):223. doi:10.1186/ar4032Ameratunga R, Longhurst H, Steele R, et al.Common variable immunodeficiency disorders, T-cell responses to SARS-CoV-2 vaccines, and the risk of chronic COVID-19.J Allergy Clin Immunol Pract. 2021;9(10):3575–3583. doi:10.1016/j.jaip.2021.06.019Genetic and Rare Diseases Information Center.Common variable immunodeficiency.MedlinePlus.Common variable immunodeficiency.National Organization for Rare Disorders.Common variable immunodeficiency.de Valles-Ibáñez G, Esteve-Solé A, Piquer M, et al.Evaluating the genetics of common variable immunodeficiency: monogenetic model and beyond.Front Immunol. 2018;9:636. doi:10.3389/fimmu.2018.00636Sperlich JM, Grimbacher B, Workman S, et al.Respiratory infections and antibiotic usage in common variable immunodeficiency.J Allergy Clin Immunol Pract.. 2018;6(1):159-168.e3. doi:10.1016/j.jaip.2017.05.024Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R.New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin.Clin Exp Immunol. 2013;174(2):203-211. doi:10.1111/cei.12178Ameratunga R, Woon S-T.Perspective: evolving concepts in the diagnosis and understanding of common variable immunodeficiency disorders(CVID).Clinic Rev Allerg Immunol. 2020;59(1):109-121. doi:10.1007/s12016-019-08765-6Cleveland Clinic.Common variable immunodeficiency (CVID).
National Institute of Allergy and Infectious Diseases.Common variable immunodeficiency (CVID).
Saikia B, Gupta S.Common variable immunodeficiency.Indian J Pediatr. 2016;83(4):338-344. doi:10.1007/s12098-016-2038-x
American Academy of Allergy Asthma & Immunology.Common variable immunodeficiency: symptoms and diagnosis.
Salzer U, Warnatz K, Peter HH.Common variable immunodeficiency - an update.Arthritis Research & Therapy. 2012;14(5):223. doi:10.1186/ar4032
Ameratunga R, Longhurst H, Steele R, et al.Common variable immunodeficiency disorders, T-cell responses to SARS-CoV-2 vaccines, and the risk of chronic COVID-19.J Allergy Clin Immunol Pract. 2021;9(10):3575–3583. doi:10.1016/j.jaip.2021.06.019
Genetic and Rare Diseases Information Center.Common variable immunodeficiency.
MedlinePlus.Common variable immunodeficiency.
National Organization for Rare Disorders.Common variable immunodeficiency.
de Valles-Ibáñez G, Esteve-Solé A, Piquer M, et al.Evaluating the genetics of common variable immunodeficiency: monogenetic model and beyond.Front Immunol. 2018;9:636. doi:10.3389/fimmu.2018.00636
Sperlich JM, Grimbacher B, Workman S, et al.Respiratory infections and antibiotic usage in common variable immunodeficiency.J Allergy Clin Immunol Pract.. 2018;6(1):159-168.e3. doi:10.1016/j.jaip.2017.05.024
Ameratunga R, Woon ST, Gillis D, Koopmans W, Steele R.New diagnostic criteria for common variable immune deficiency (CVID), which may assist with decisions to treat with intravenous or subcutaneous immunoglobulin.Clin Exp Immunol. 2013;174(2):203-211. doi:10.1111/cei.12178
Ameratunga R, Woon S-T.Perspective: evolving concepts in the diagnosis and understanding of common variable immunodeficiency disorders(CVID).Clinic Rev Allerg Immunol. 2020;59(1):109-121. doi:10.1007/s12016-019-08765-6
Cleveland Clinic.Common variable immunodeficiency (CVID).
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