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Selective Isotype Immunodeficiency

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Primary and Secondary Immunodeficiency

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Abstract

Common variable immunodeficiency (CVID) is a disorder characterized by patients with recurrent infections who have reduced levels of total IgG and reduced levels of either IgA or IgM. In contrast, selective immunoglobulin deficiency is a disease in which patients have a reduced level of IgA, IgM, or one of the four subtypes of IgG, while their total IgG is normal. The most common primary antibody deficiency is a selective IgA deficiency. The implications of an IgA deficiency range from no clinical significance, where the deficiency was found as an incidental finding, such as during testing for celiac disease, to significant autoimmunity and immunodeficiency. Selective IgG deficiency is a decreased level of one of the four subtypes of IgG in a patient with recurrent infections and a normal serum level of total IgG with no other immunological aberrations. It is a distinct disorder from specific antibody deficiency (SAD) in which a patient has normal levels of immunoglobulins, including IgG subclasses, but poor responses to polysaccharide antigens. Selective IgG deficiency, similar to selective IgA deficiency, may be seen in patients without any clinical consequences; however, many present with significant immunodeficiency and atopic disorders. Patients with selective immunoglobulin deficiency require close clinical monitoring to ensure there is no progression of their disease state. Severe forms may require prophylactic antibiotics and possibly immunoglobulin replacement therapy.

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Authors and Affiliations

  1. New York Allergy and Sinus Center, Glendale, NY, USA

    Robert Tamayev

  2. Department of Allergy and Immunology, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, NY, USA

    Jenny Shliozberg

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  1. College of Medicine, University of Cincinnati, Cincinnati, OH, USA

    Jonathan A. Bernstein

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Tamayev, R., Shliozberg, J. (2021). Selective Isotype Immunodeficiency. In: Bernstein, J.A. (eds) Primary and Secondary Immunodeficiency. Springer, Cham. https://doi.org/10.1007/978-3-030-57157-3_6

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