TY  - JOUR
T1  - Targeted treatments for immune dysregulation in inborn errors of immunity
AU  - Makkoukdji, Nadia
AU  - Pundit, Valishti
AU  - Wyke, Matthew
AU  - Giraldo, Jheison
AU  - Satnarine, Travis
AU  - Kleiner, Gary I.
AU  - Gans, Melissa
Y1  - 2024///
JO  - Exploration of Immunology
VL  - 4
IS  - 2
SP  - 218
EP  - 237
DO  - 10.37349/ei.2024.00138
UR  - https://www.explorationpub.com/Journals/ei/Article/1003138
AB  - Primary immune regulatory disorders (PIRDs) constitute a subset of inborn errors of immunity and are characterized by lymphoproliferation, autoimmunity, malignancy, and infection. Unlike classical primary immune deficiencies, initial symptoms of PIRDs can manifest as autoimmunity such as cytopenias or enteropathy, which can often prove resistant to conventional treatments and occur years prior to the onset of infectious complications. Raising awareness about PIRDs among specialists and adopting a multidisciplinary approach is crucial for early diagnosis, intervention, and potential prevention of severe organ damage. Significant progress has been made in identifying several PIRDs, which has contributed to a more comprehensive comprehension of their underlying immunological mechanisms. This knowledge has paved the way for targeted therapies focusing on specific molecules, which tend to offer superior disease control compared to traditional immunosuppressants. This review, informed by the latest literature, explores prevalent PIRDs, detailing their clinical manifestations and recent advancements in treatment modalities.
ER  -