Autoimmune diseases occur when a sustained, specific, adaptive immune response is generated against self-components, and results in tissue damage or dysfunction. It is now clear that an autoimmune component is a feature of many human diseases. Indeed, there are some estimates that autoimmune diseases afflict more than 3% of Western populations, and imposes a significant personal and economic burden on individuals and nations. They probably affect more commonly women than men, and have peak incidence in the third to sixth decades. This chapter will illustrate many of the principles unifying various autoimmune states, and will present a conceptual framework within which to understand their aetiology, pathogenesis, and pathology. The rapid advances in knowledge being made in this group of disorders predict that disease mechanisms will soon be more clearly understood, and will greatly impact therapeutics.