Abstract
Primary immunodeficiency disorders (PIDs) are genetically predetermined conditions of innate or adaptive immune system dysregulation. Susceptibility toward infection has been a long-standing, often diagnosis-requiring, feature. More recently, autoimmunity as a central pathology in PIDs has been described. Our growing ability to link unique genetic defects with downstream mechanisms that drive hyperinflammation and loss of self-tolerance is extremely powerful. Ultimately, successful management of the PID patient requires not only prevention of infection, but early detection and treatment of autoimmune complications through the use of mechanism-based therapies, which will directly address the immune dysregulation at play.
| Original language | English |
|---|---|
| Title of host publication | Translational Immunology: Mechanisms and Pharmacologic Approaches |
| Publisher | Elsevier Inc. |
| Pages | 45-81 |
| Number of pages | 37 |
| ISBN (Print) | 9780128015773 |
| DOIs | |
| Publication status | Published - Dec 19 2015 |
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Keywords
- Autoimmunity
- Common variable
- Congenital
- Hyperinflammation
- Immunodeficiency
- Primary
ASJC Scopus subject areas
- Medicine(all)
- Immunology and Microbiology(all)
Cite this
Congenital Immunodeficiency Diseases : Crossroad of Infection, Autoimmunity, and Hyperinflammation. Crossroad of Infection, Autoimmunity, and Hyperinflammation. / Farmer, J.; Csomos, K.; Bacchetta, R.; Walter, J. E.
Translational Immunology: Mechanisms and Pharmacologic Approaches. Elsevier Inc., 2015. p. 45-81.Research output: Chapter in Book/Report/Conference proceeding › Chapter
}
TY - CHAP
T1 - Congenital Immunodeficiency Diseases
T2 - Crossroad of Infection, Autoimmunity, and Hyperinflammation. Crossroad of Infection, Autoimmunity, and Hyperinflammation
AU - Farmer, J.
AU - Csomos, K.
AU - Bacchetta, R.
AU - Walter, J. E.
PY - 2015/12/19
Y1 - 2015/12/19
N2 - Primary immunodeficiency disorders (PIDs) are genetically predetermined conditions of innate or adaptive immune system dysregulation. Susceptibility toward infection has been a long-standing, often diagnosis-requiring, feature. More recently, autoimmunity as a central pathology in PIDs has been described. Our growing ability to link unique genetic defects with downstream mechanisms that drive hyperinflammation and loss of self-tolerance is extremely powerful. Ultimately, successful management of the PID patient requires not only prevention of infection, but early detection and treatment of autoimmune complications through the use of mechanism-based therapies, which will directly address the immune dysregulation at play.
AB - Primary immunodeficiency disorders (PIDs) are genetically predetermined conditions of innate or adaptive immune system dysregulation. Susceptibility toward infection has been a long-standing, often diagnosis-requiring, feature. More recently, autoimmunity as a central pathology in PIDs has been described. Our growing ability to link unique genetic defects with downstream mechanisms that drive hyperinflammation and loss of self-tolerance is extremely powerful. Ultimately, successful management of the PID patient requires not only prevention of infection, but early detection and treatment of autoimmune complications through the use of mechanism-based therapies, which will directly address the immune dysregulation at play.
KW - Autoimmunity
KW - Common variable
KW - Congenital
KW - Hyperinflammation
KW - Immunodeficiency
KW - Primary
UR - http://www.scopus.com/inward/record.url?scp=84960806122&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84960806122&partnerID=8YFLogxK
U2 - 10.1016/B978-0-12-801577-3.00002-2
DO - 10.1016/B978-0-12-801577-3.00002-2
M3 - Chapter
SN - 9780128015773
SP - 45
EP - 81
BT - Translational Immunology: Mechanisms and Pharmacologic Approaches
PB - Elsevier Inc.
ER -