A New Classification System for IgG4 Autoantibodies.

摘要: IgG4 autoimmune diseases are characterized by the presence of antigen-specific autoantibodies subclass and contain well-characterized such as muscle-specific kinase myasthenia gravis, pemphigus, thrombotic thrombocytopenic purpura. In recent years, several new were identified, now 14 antigens targeted have been described. The is considered immunologically inert functionally monovalent due to structural differences compared other IgG subclasses. usually arises after chronic exposure antigen competes with antibody species, thus "blocking" their pathogenic effector mechanisms. Accordingly, in context autoimmunity, pathogenicity associated blocking enzymatic activity or protein-protein interactions target antigen. Pathogenicity has not yet systematically analyzed diseases. Here, we establish a modified classification system based on Witebsky's postulates determine diseases, review characteristics mechanisms these disorders, also investigate contribution entities pathophysiology additional As result, three classes emerge: class I where validated use subclass-specific animal models and/or vitro pathogenicity; II highly suspected but lack validation specific antibodies models; III insufficient data mechanism multivalent binding. Five out I, five II, four III. Antibodies subclasses immunoglobulin present could contribute