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Inborn errors of immunity (primary immunodefciencies)

Kim, V.H.D., Upton, J.E.M., Derfalvi, B. et al. Inborn errors of immunity (primary immunodeficiencies). Allergy Asthma Clin Immunol 20 (Suppl 3), 76 (2024). https://doi.org/10.1186/s13223-024-00938-z

Abstract

Primary immunodefciencies (PID), now often referred to as inborn errors of immunity (IEI), are a large heterogeneous group of disorders that result from defciencies in immune system development and/or function. IEIs can be broadly classifed as disorders of adaptive immunity (e.g., combined or humoral immunodefciencies) or of innate immunity (e.g., phagocyte and complement disorders). Although the clinical manifestations of IEIs are highly variable, traditionally many disorders involve an increased susceptibility to infection. Research in recent years has underscored how IEI can present with features other than infection such as: severe atopy, autoimmunity, autoinfammation, lymphoproliferation, and/or malignancy resulting from immune dysregulation. Early consultation with a clinical immunologist is essential, as timely diagnosis and treatment are imperative for preventing signifcant disease-associated morbidity and mortality. The treatment of IEIs is complex and generally requires both supportive and defnitive strategies, including but not limited to, immunoglobulin replacement therapy, antibiotic prophylaxis, immune response modifers, and hematopoietic stem cell transplantation. This article provides an overview of the major categories of IEIs and strategies for the appropriate diagnosis and management of these disorders.

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https://aacijournal.biomedcentral.com/counter/pdf/10.1186/s13223-024-00938-z.pdf
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