Abstract
The immune system protects against infection with microorganisms. This system is remarkable in both its complexity and its effectiveness. Without a functioning immune system, humans cannot survive past the first few months of life. However, as extraordinary this system may be, until the most recent century, infections were by far the most common cause of death among humans as microorganisms kept the average life expectancy to about 25–30 years. The increased life expectancy currently seen reflects progress in control of infectious diseases including improved hygiene, vaccines, and antimicrobial drugs. Casanova and Abel have proposed that, by definition, humans who die of infection are immunodeficient and that recent medical progress has masked widespread inherited defects in immunity. A better understanding of the weaknesses and limitations of the immune system will lead to approaches to assist and improve it. This chapter addresses major syndromes of immune dysfunction. These include diseases of deficient immunity, hyperactive immunity (hypersensitivity), and dysregulated immunity (autoimmune diseases). To understand the pathophysiology of these syndromes, one must have an understanding of how the normal immune system functions. The chapter presents a brief summary of the important cells and molecules of the immune system.
Published Version
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