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 |
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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 |
Keywords
- Autoimmunity
- Common variable
- Congenital
- Hyperinflammation
- Immunodeficiency
- Primary
ASJC Scopus subject areas
- Medicine(all)
- Immunology and Microbiology(all)