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The Relationship of X-Linked Primary Immune Deficiencies and Autoimmunity

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Abstract

It is well-known that autoimmunity is significantly more prevalent in females. Growing evidence indicates that genes located on the X chromosome may play a role in autoimmunity and immune dysregulation, as also indicated by the frequent association of autoimmune phenomena in patients with X-linked primary immune deficiencies (PIDs). Hence, this group of genetic disorders is of particular interest to study PID-causing genes in the setting of more complex autoimmune disorders. This review focuses on the mechanisms leading to the autoimmune phenomena that are associated with the different X-linked PIDs, and on the intriguing interplay between immune dysregulation and immune deficiency in this unique setting.

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Authors and Affiliations

  1. Division of Immunology, Children’s Hospital, Harvard Medical School, 9th Floor, One Blackfan Circle, Boston, MA, 02115, USA

    Itai M. Pessach

  2. Talpiot Medical Leadership Program, Sheba Medical Center, Safra Children’s Hospital, Tel-Hashomer, 52621, Israel

    Itai M. Pessach

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Pessach, I.M. The Relationship of X-Linked Primary Immune Deficiencies and Autoimmunity. Curr Allergy Asthma Rep 10, 311–319 (2010). https://doi.org/10.1007/s11882-010-0127-x

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