The front line of research into immunoglobin G4-related disease - Do autoantibodies cause immunoglobin G4-related disease?

Abstract

IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. Although IgG4-RD is attracting strong attention as a new clinical entity, the pathogenesis of IgG4-RD and the roles of IgG4 are still unknown. Recently, self-antigens including Laminin 511 E8, Galectin-3 and Annexin A11, have been reported from one to the next as autoantigens which may be involved in the pathogenesis of IgG4-RD. In this review, we describe up-to-date information on the research of this emerging disease entity. Moreover, we discuss the pathogenesis of IgG4-RD by focusing on recent reports concerning autoantibodies.

Keywords:

• IgG4-RD
• autoantibody
• autoantigen
• innate immunity
• acquired immunity
• pathogenesis
• autoimmunity

Acknowledgments

We sincerely thank the many contributing researchers and collaborators who participated in the all Japan IgG4-RD Research Group.

Conflict of interest

None

Additional information

Funding

This work was supported by Intractable Diseases and Health and Labor Sciences Research Grants from the Ministry of Health, Labor and Welfare of Japan.