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Selective IgA Deficiency

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Humoral Primary Immunodeficiencies

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Selective IgA deficiency (IgAD) is the most common primary immunodeficiency with a wide range of clinical symptoms including respiratory and gastrointestinal infections, autoimmunity, allergic conditions, and, in some cases, progression to the more severe antibody deficiency, common variable immunodeficiency (CVID). Genetics, intrinsic defects in lymphocytes, and impairment of the cytokine profile are all thought to contribute to IgAD pathogenesis, but the underlying cause of the diseases still remains unknown. This, together with a wide range of clinical manifestations in the patients and the association between IgAD and other immune disorders, presents a challenge for clinicians and researchers in both diagnosing and treating IgAD.

In this chapter we have summarized the epidemiology, pathogenesis, clinical phenotype, diagnosis, prognosis, and treatment of IgAD.

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Authors and Affiliations

  1. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden

    K. Sofia Appelberg, Hassan Abolhassani & Lennart Hammarström

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  1. K. Sofia Appelberg
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  2. Hassan Abolhassani
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  3. Lennart Hammarström
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  1. Careggi Hospital, Department of Clinical and Experimental Medicine, University of Florence, Firenze, Italy

    Mario Milco D'Elios

  2. University Medical Centre Freiburg, Department of Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency, Freiburg, Germany

    Marta Rizzi

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Sofia Appelberg, K., Abolhassani, H., Hammarström, L. (2019). Selective IgA Deficiency. In: D'Elios, M., Rizzi, M. (eds) Humoral Primary Immunodeficiencies. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-91785-6_16

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