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Common Variable Immunodeficiency (CVID)

By

James Fernandez

, MD, PhD, Cleveland Clinic Lerner College of Medicine at Case Western Reserve University

Reviewed/Revised Jan 2023
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Common variable immunodeficiency (acquired or adult-onset hypogammaglobulinemia) is characterized by low immunoglobulin (Ig) levels with phenotypically normal B cells that can proliferate but do not develop into Ig-producing cells. Patients have recurrent sinopulmonary infections. Diagnosis is based mainly on serum Ig levels. Treatment includes prophylactic IgG replacement therapy and antibiotics for infection.

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Symptoms and Signs of CVID

Patients with CVID have recurrent sinopulmonary infections causing sinus congestion and pressure, cough, shortness of breath, chest pain, mucus production, fevers, chills, and/or lymphadenopathy.

Diagnosis of CVID

  • Measurement of serum immunoglobulin (Ig) and antibody titers

  • Flow cytometry for T-cell and B-cell subsets

  • Serum protein electrophoresis

Diagnosis of common variable immunodeficiency is suggested by recurrent sinopulmonary infections and requires all of the following:

  • Low (at least 2 standard deviations below the mean) levels of IgG

  • Low levels of IgA, IgM, or both

  • Impaired response to immunizations (usually both protein and polysaccharide vaccines)

  • Exclusion of other immunodeficiency disorders

Antibody or autoantibody levels should not be measured if patients have been treated with IV immune globulin (IVIG) within the previous 6 months because any detected antibodies may be from the IVIG.

Spirometry, complete blood count, liver tests, and a basic metabolic panel are recommended yearly to check for associated disorders. If lung function changes, CT should be done.

Because mutations are usually sporadic, screening relatives is not recommended unless there is a significant family history of CVID.

Treatment of CVID

  • Prophylactic immune globulin (IgG) replacement therapy

  • Antibiotics for infections

  • Disease-specific treatment for comorbid immunodeficiency disorders such as granulomatous lung disease or associated autoimmune disease

Rituximab, vedolizumab, tumor necrosis factor (TNF)-alpha inhibitors (eg, etanercept, infliximab), corticosteroids, and/or other treatments may be required to treat complications such as autoimmune disorders, immune enteropathy, lymphoid interstitial pneumonia, and granulomatous inflammation.

Treatment reference

  • 1. Milito C, Pulvirenti F, Cinetto F, et al: Double-blind, placebo-controlled, randomized trial on low-dose azithromycin prophylaxis in patients with primary antibody deficiencies. J Allergy Clin Immunol 144:584–593. e7, 2019. doi: 10.1016/j.jaci.2019.01.051

Drugs Mentioned In This Article

Drug Name Select Trade
RIABNI, Rituxan, RUXIENCE, truxima
Entyvio
Enbrel
AVSOLA, INFLECTRA, Remicade, RENFLEXIS, Zymfentra
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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