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Autoimmunity in Immunodeficiency

  • AUTOIMMUNITY (TK TARRANT, SECTION EDITOR)
  • Published:
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Abstract

Primary immunodeficiencies (PID) comprise a diverse group of clinical disorders with varied genetic defects. Paradoxically, a substantial proportion of PID patients develop autoimmune phenomena in addition to having increased susceptibility to infections from their impaired immunity. Although much of our understanding comes from data gathered through experimental models, there are several well-characterized PID that have improved our knowledge of the pathways that drive autoimmunity. The goals of this review will be to discuss these immunodeficiencies and to review the literature with respect to the proposed mechanisms for autoimmunity within each put forth to date.

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Conflict of Interest

Krista Todoric declares that she has no conflict of interest.

Jessica B. Koontz declares that she has no conflict of interest.

Daniel Mattox declares that he has no conflict of interest.

Teresa K. Tarrant has served as a consultant for Roche.

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Authors and Affiliations

  1. Division of Allergy and Immunology, Dept of Pediatrics, University of North Carolina Hospitals, Chapel Hill, NC, 27599, USA

    Krista Todoric

  2. Thurston Arthritis Research Center, University of North Carolina, Chapel Hill, NC, 27599, USA

    Jessica B. Koontz & Daniel Mattox

  3. Thurston Arthritis Research Center, Divisions of Rheumatology & Allergy and Immunology, Dept of Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, 27599, USA

    Teresa K. Tarrant

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  1. Krista Todoric
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  2. Jessica B. Koontz
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  4. Teresa K. Tarrant
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Correspondence to Teresa K. Tarrant.

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Todoric, K., Koontz, J.B., Mattox, D. et al. Autoimmunity in Immunodeficiency. Curr Allergy Asthma Rep 13, 361–370 (2013). https://doi.org/10.1007/s11882-013-0350-3

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