[Antibodydeficiencies Epidemiology, Clinical manifestation, Diagnostics and Therapy]

Jandus, Peter; Bitzenhofer-Grüber, Michaela (2022). [Antibodydeficiencies Epidemiology, Clinical manifestation, Diagnostics and Therapy]. Therapeutische Umschau, 79(6), pp. 279-283. Hogrefe 10.1024/0040-5930/a001361

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Antibodydeficiencies Epidemiology, Clinical manifestation, Diagnostics and Therapy Abstract. Primary Immune Deficiencies (PID) are caused by a genetically induced malformation/dysfunction of the immune system. Leading symptoms include susceptibility to infection, autoimmune diseases, lymphoproliferative, allergic as well as malignant diseases. They can be divided into ten main groups, including the primary antibody deficiency syndromes (PAD) in adults. The most well-known PADs include the variable immunodeficiency syndrome (CVID), Bruton's agammaglobulinaemia, IgG subclass deficiencies, immunoglobulin A deficiency, Antibody deficiency and transient childhood hypogammaglobulinaemia. Secondary hypogammaglobulinaemia by medicinal products, haematological diseases, malignancies and infections should be excluded. Delayed diagnosis of CVID is associated with a significant increase in morbidity and an increase in mortality. In addition to vaccinations, immunoglobulin replacement therapy is used therapeutically.

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