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Late-Onset Combined Immunodeficiencies (LOCID)

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Humoral Primary Immunodeficiencies

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

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Abstract

The term common variable immunodeficiencies (CVIDs) is applied to a heterogeneous group of primary immunodeficiencies with hypogammaglobulinemia and defective B cell maturation or specific antibody formation. This diagnosis has evolved since first coined in 1971, based on proposed and subsequently revised clinical and immunological criteria. The genetic base of this condition is poorly defined, and only 15–30% of cases seem to be in relation with clear monogenic inheritance. Clinically, CVID can present with infections only or with other disease-related complications. Although it is accepted that CVID could arise as consequence of a B cell intrinsic defect, a certain component of T cell deficiency has been largely appreciated. Therefore, several refinements of the CVID definition and criteria have been proposed trying to exclude from this diagnosis late-onset combined immunodeficiencies (LOCID) that require proper diagnostic and clinical management approaches. This chapter tries to summarize the evolving concepts around the LOCID vs. CVID dilemma and the current perspective.

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Authors and Affiliations

  1. Coordinador del Servicio de Inmunología, Hospital Universitario La Paz-IdiPAZ, Madrid, Spain

    Eduardo López-Granados

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  1. Eduardo López-Granados
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Correspondence to Eduardo López-Granados .

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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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López-Granados, E. (2019). Late-Onset Combined Immunodeficiencies (LOCID). 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_4

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  • DOI: https://doi.org/10.1007/978-3-319-91785-6_4

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  • Online ISBN: 978-3-319-91785-6

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