Inmunodeficiencias primarias: inmunopatogenia, infecciones asociadas y estrategias terapéuticas

Primary immunodeficiencies disorders (PID) are a group of genetic defects that affect both innate and adaptive immune response. PID are associated with: 1) absence of cellular components, 2) impaired connection among components of the immune response, as well as in the recognition of foreign, self or modified antigens, and 3) inappropriate modulation of effectors mechanisms either by inability to reach affected tissues, to promote proinflammatory and regulatory polarization, or perform the clearance of invading agents. PID not only increases the susceptibility to infections by different microorganisms, but also alters the homeostatic mechanisms and immune surveillance, that prevent autoinflammatory and neoplastic diseases. This review describes some of these defects, its consequences and the initial approach to the study and management of recurrent infections.