The natural history and pathogenesis of IgG4-related disease

Abstract

(IgG4-RD) is a chronic fibroinflammatory condition that is relatively recently recognized and defined as a distinct clinical entity that can affect virtually every organ system. Although the concept of IgG4-RD is rapidly evolving, the immunopathogenic mechanism underlying this disease is largely unknown. Autoimmunity has been suggested as the most possible pathogenetic cause, but autoantibodies against disease-specific antigens have not been determined. The behavior of the IgG4 antibody, as well as the functional properties of different B cell subsets involved in the inflammatory response, remains to be elucidated. In this report we will address 1) the proposed involvement of Helicobacter pylori and 2) the phenotype or functional properties of various peripheral B cell subsets in the blood of patients with IgG4-RD.