ABSTRACT

Autoimmune disease results when the adaptive immune response is directed against self antigens. The particular pathology of the disease depends on the nature of the self antigen and the type of immune response that is mounted against it. In the type II autoimmune diseases (see Fig. 40.1) the pathology is caused by autoantibodies that interact with self antigens in the extracellular matrix or on cell surfaces. In myasthenia gravis, for example, autoantibodies against the acetylcholine receptor on skeletal muscle block its function at the neuromuscular junction and cause paralysis (see Case 40), whereas in Graves’ disease an autoantibody against the receptor for thyroid-stimulating hormone acts as an agonist, stimulating receptor activity and causing hyperthyroidism. In the autoimmune disease pemphigus vulgaris, the self-reactive agent is an autoantibody against a structural protein of the epidermal cells of the skin. Its actions result in the skin cells coming apart from each other; the affected skin blisters and is destroyed.