The relationship of x-linked primary immune deficiencies and autoimmunity

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Pages (from-to)311-319
Number of pages9
JournalCurrent Allergy and Asthma Reports
Issue number5
StatePublished - Sep 2010
Externally publishedYes


  • Autoimmunity
  • Primary immune deficiencies
  • X-linked


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