title: Unbalanced Immune System: Immunodeficiencies and Autoimmunity.
abstract: Increased risk of developing autoimmune manifestations has been identified in different primary immunodeficiencies (PIDs). In such conditions, autoimmunity and immune deficiency represent intertwined phenomena that reflect inadequate immune function. Autoimmunity in PIDs may be caused by different mechanisms, including defects of tolerance to self-antigens and persistent stimulation as a result of the inability to eradicate antigens. This general immune dysregulation leads to compensatory and exaggerated chronic inflammatory responses that lead to tissue damage and autoimmunity. Each PID may be characterized by distinct, peculiar autoimmune manifestations. Moreover, different pathogenetic mechanisms may underlie autoimmunity in PID. In this review, the main autoimmune manifestations observed in different PID, including humoral immunodeficiencies, combined immunodeficiencies, and syndromes with immunodeficiencies, are summarized. When possible, the pathogenetic mechanism underlying autoimmunity in a specific PID has been explained.
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Giuliana
authors.initials: G
authors.lastname: Giardino
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Vera
authors.initials: V
authors.lastname: Gallo
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Rosaria
authors.initials: R
authors.lastname: Prencipe
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Gaudino
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Romano
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: De Cataldis
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Lorello
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Loredana
authors.initials: L
authors.lastname: Palamaro
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Di Giacomo
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Donatella
authors.initials: D
authors.lastname: Capalbo
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Emilia
authors.initials: E
authors.lastname: Cirillo
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: D'Assante
authors.affiliation: Department of Translational Medical Sciences, Federico II University of Naples , Naples , Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Pignata
keywords: autoimmune hemolytic anemia; autoimmunity; immune thrombocytopenia; immunodeficiency; systemic lupus erytematous