Primary Immunodeficiency Diseases
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Primary immunodeficiency diseases (PIDs) are a heterogeneous group of monogenic disorders in which part of the immune system is deficient or does not function properly. Patients with PID commonly present with recurrent infections, which explains the importance of the immune system in protection from organisms. PIDs are classified according to the affected component of the immune system into innate immunity disorders and adaptive immunity disorders. Early diagnosis and aggressive management ranging from medical treatment to stem cell transplantation are crucial to decrease disease-associated morbidity and mortality. In this review, we will provide a detailed overview of classification, diagnosis, and treatments of PIDs, with particular attention to the critical care management of the common complications of these rare diseases.
KeywordsPrimary immunodeficiency diseases Innate immunodeficiencies Humoral immunodeficiencies Cellular immunodeficiencies Hematopoietic cell transplantation Gene therapy
Conflict of Interest
The authors declare that they have no conflict of interest.
- 8.Leiding JW, Holland SM. Chronic granulomatous disease. In: Adam MP, et al., editors. GeneReviews((R)). Seattle: University of Washington; 1993.Google Scholar
- 17.Sakaguchi S, et al. Pillars article: immunologic self-tolerance maintained by activated T cells expressing IL-2 receptor alpha-chains (CD25). Breakdown of a single mechanism of self-tolerance causes various autoimmune diseases. J Immunol. 1995. J Immunol. 2011;186(7):3808–21.Google Scholar