Published online Mar 12, 2016. doi: 10.5499/wjr.v6.i1.16
Peer-review started: October 3, 2015
First decision: November 24, 2015
Revised: December 23, 2015
Accepted: January 5, 2016
Article in press: January 7, 2016
Published online: March 12, 2016
IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease with multiple organ disorders. Recently, in IgG4-RD, increased circulating plasmablasts have been found. The subsets of plasmablasts are negative for RP105 (CD180). A large population of B cells lacking RP105 (RP105-negative B cells) are found in patients with active with systemic lupus erythematosus and other systemic autoimmune diseases, including dermatomyositis, and Sjögren’s syndrome. In other conditions, such as neuromyelitis optica, Kawasaki’s disease, primary biliary cirrhosis and aging, RP105 expression on B cells and monocytes also alters. We review the basic science and clinical significance of RP105-negative B cells including plasmablasts in various immune-based diseases. RP105-negative B cells, especially plasmablasts, play crucial roles in both systemic and organ-specific autoimmune and inflammatory disorders.
Core tip: RP105 (CD180) is associated with B cell function, survival and death. RP105-negative B cells, especially plasmablasts, take part in pathophysiology of various immune-based diseases.