title: Autoimmunity in immunodeficiency.
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.
authors.affiliation: Division of Allergy and Immunology, Dept of Pediatrics, University of North Carolina Hospitals, Chapel Hill, NC 27599, USA.
authors.forename: Krista
authors.initials: K
authors.lastname: Todoric
authors.forename: Jessica B
authors.initials: JB
authors.lastname: Koontz
authors.forename: Daniel
authors.initials: D
authors.lastname: Mattox
authors.forename: Teresa K
authors.initials: TK
authors.lastname: Tarrant
mesh_terms: D015551:Autoimmunity / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000276:immunology*; D007239:Infections / Q000276:immunology; D015398:Signal Transduction / Q000502:physiology