What is it an immunodeficiency?
Immune deficiencies are characterised by a temporary or permanent impairment or even a malfunction of the defence system, in other words: impairment/malfunction of the ability to protect against pathogenic organisms. As a result, infectious diseases occur more frequently causing more severe symptoms. Immune deficiencies are frequently associated with autoimmune phenomena. As there is a malfunction of the defence system, the body's own tissues are wrongly fought as foreign matter. This means that the body's own tissues are no longer tolerated. Hence, the body fails to discriminate between self and non-self.
Immune deficiencies can be genetically pre-programmed (primary) or be the result of other diseases (secondary). The causes of secondary immune deficiencies are equally varied. They develop, for example, following infections (HI-Virus [AIDS], measles) or during certain drug therapies (immunosuppressants, cytostatics, corticosteroids, etc.).
Both primary and secondary immune deficiencies can manifest themselves already in childhood, or not until adulthood.
The diagnosis of an immune deficiency often occurs years after the appearance of the first symptoms, as it was not previously considered.
Depending on which defence processes are limited in their function, immune deficiencies can be categorised into various groups. Examples are immunoglobulin deficiencies, complement deficiencies, B-cell deficiencies, T-cell deficiencies, or deficiencies in the "scavenger cells" (phagocytic cells).
The frequency of these congenital immune deficiencies is not exactly known. They vary between 1:10,000 and 1:500,000 according to the type and region. There are already over 200 different types of known primary immune deficiencies.