Chapter 342: Introduction to the Immune System

DEFINITIONS

• Adaptive immune system—recently evolved system of immune responses mediated by T and B lymphocytes. Immune responses by these cells are based on specific antigen recognition by clonotypic receptors that are products of genes that rearrange during development and throughout the life of the organism. Additional cells of the adaptive immune system include various types of antigen-presenting cells (APCs).

• Antibody—B cell–produced molecules encoded by genes that re-arrange during B cell development consisting of immunoglobulin heavy and light chains that together form the central component of the B cell receptor (BCR) for antigen. Antibody can exist as B cell–surface antigen-recognition molecules or as secreted molecules in plasma and other body fluids.

• Antigens—foreign or self-molecules that are recognized by the adaptive and innate immune systems resulting in immune cell triggering, T cell activation, and/or B cell antibody production.

• Antimicrobial peptides—small peptides <100 amino acids in length that are produced by cells of the innate immune system and have anti-infectious agent activity.

• Apoptosis—the process of programmed cell death whereby signaling through various “death receptors” on the surface of cells (e.g., tumor necrosis factor [TNF] receptors, CD95) leads to a signaling cascade that involves activation of the caspase family of molecules and leads to DNA cleavage and cell death. Apoptosis, which does not lead to induction of inordinate inflammation, is to be contrasted with cell necrosis, which does lead to induction of inflammatory responses.

• Autoimmune diseases—diseases such as systemic lupus erythematosus and rheumatoid arthritis in which cells of the adaptive immune system such as autoreactive T and B cells become overreactive and produce self-reactive T cell and antibody responses.

• Autoinflammatory diseases—hereditary disorders such as hereditary periodic fevers (HPFs) characterized by recurrent episodes of severe inflammation and fever due to mutations in controls of the innate inflammatory response, i.e., the inflammasome (see below and Table 342-6). Patients with HPFs also have rashes and serosal and joint inflammation, and some can have neurologic symptoms. Autoinflammatory diseases are different from autoimmune diseases in that evidence for activation of adaptive immune cells such as autoreactive B cells is not present.

• Autophagy—lysosomal degradation pathway mechanism of cells to dispose of intracellular debris and damaged organelles. Autophagy by cells of the innate immune system is used to control intracellular infectious agents such as mycobacteria, in part by initiation of phagosome maturation and enhancing major histocompatibility complex (MHC) class II antigen presentation to CD4 T cells.

• B cell receptor for antigen—complex of surface molecules that rearrange during postnatal B cell development, made up of surface immunoglobulin (Ig) and associated Ig αβ chain molecules that recognize nominal antigen via Ig heavy- and light-chain variable regions, and signal the B cell to terminally differentiate to make antigen-specific antibody.

• B lymphocytes—bone marrow-derived or bursal-equivalent lymphocytes that express surface immunoglobulin (the BCR for antigen) and secrete specific antibody after interaction with antigen.

• B regulatory cells—a population of suppressive B cells that aid in the ...