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Management of Humoral Primary Immunodeficiencies in Adults

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Humoral Primary Immunodeficiencies

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

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Abstract

Immunoglobulin replacement therapy is the cornerstone for the management of humoral primary immunodeficiencies as its major goal is the minimization of infective complications. Clinical phenotype and the presence of complications should guide the dosage of immunoglobulin replacement therapy and the choice between intravenous (IVIg) and subcutaneous (SCIg) route of administration. Mild infusion reactions are common with IVIg, whereas local pain, swelling, and erythema represent the major complaints with SCIg. Severe adverse reactions are rare and mainly associated to high-dose IVIg and comprise anaphylaxis, aseptic meningitis, and thrombosis.

Antibiotic prophylaxis is sometimes used in these patients, but standard protocols are still lacking. As unusual microorganisms and antibiotic resistance are common, an antibiogram should be obtained before initiating an empiric therapy.

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Authors and Affiliations

  1. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

    Gianfranco Vitiello, Giacomo Emmi & Boaz Palterer

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  1. Gianfranco Vitiello
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  2. Giacomo Emmi
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Correspondence to Giacomo Emmi .

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Editors and Affiliations

  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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Vitiello, G., Emmi, G., Palterer, B. (2019). Management of Humoral Primary Immunodeficiencies in Adults. 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_21

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  • DOI: https://doi.org/10.1007/978-3-319-91785-6_21

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