Common variable immune deficiency and autoimmunity

Daniel Brandt, M. Eric Gershwin

Research output: Contribution to journalArticle

108 Scopus citations

Abstract

Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by various degrees of hypogammaglobulinemia. Similar to many immunodeficiency disorders, autoimmunity is common with an association with autoimmune cytopenias, a sarcoidosis-like disorder and inflammatory bowel disease. Recent efforts have characterized selective immunological defects and genetic associations in CVID and demonstrate an increased tendency towards loss of tolerance. The mainstay of treatment of autoimmune disease in such patients is often high dose IVIG and corticosteroids, although other therapies, including TNF-α antagonists, have been reported. While the etiology of increased autoimmunity in CVID remains elusive, certain genetic predispositions in combination with repeated antigen exposure and overall immune dysregulation inherent in CVID likely play a significant role.

Original languageEnglish (US)
Pages (from-to)465-470
Number of pages6
JournalAutoimmunity Reviews
Volume5
Issue number7
DOIs
StatePublished - Aug 2006

Keywords

  • Autoimmunity
  • Class-switched memory B cells
  • Common variable immunodeficiency
  • Cytopenia
  • Granulomatous disease

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

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