Host defense from infection depends upon a complex integrated system of physical barriers (e.g., skin, stomach acid, and mucociliary clearance), innate immunity (e.g., phagocytic cells, natural killer cells, complement) and adaptive immunity (B and T lymphocytes). An individual may have deficiencies of one or more components of host defense, but no individual is defenseless. Because each functional compartment of the immune system plays a specialized role in host defense, defects in specific functions lead to increased susceptibility to specific pathogens. The key to understanding the susceptibility of a particular patient is to understand the specific host defense defects of that patient. This chapter will briefly review the components of host defense and the types of infections that are most likely to occur with specific defects in those defense mechanisms. Following that will be descriptions of the infections that occur in patients with a variety of primary and secondary immune deficiency disorders, with the intent of providing illustrative examples.
B. Overview of Host DefensesHost defense depends upon physical barriers, as well as the immune system. In conceptual terms, the components of the immune system can be divided into two compartments -innate and adaptive -with fundamentally different modes of action (Table 1). Innate host defense mechanisms are rapid (minutes to hours), depend upon patterned responses to pathogens (e.g., by phagocytic cells, complement) and do not improve with repeated exposure to one or many pathogens. In contrast, adaptive immune mechanisms are slow (days), depend upon very specific responses to individual antigens (e.g., by B and T lymphocytes), and improve with repeated exposure to an individual antigen. Successfully integrated and functioning together, physical barriers and the components of innate and adaptive immunity form a critical homeostatic mechanism necessary for the host's defense against infection and the generation of normal inflammatory responses (1, 2).