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World J Rheumatol. Mar 12, 2016; 6(1): 16-22
Published online Mar 12, 2016. doi: 10.5499/wjr.v6.i1.16
Roles of plasmablasts in IgG4-related disease and various immune-based diseases
Syuichi Koarada, Yoshifumi Tada
Syuichi Koarada, Yoshifumi Tada, Department of Rheumatology, Faculty of Medicine, Saga University, Saga 849-8501, Japan
Author contributions: All author equally contributed to this paper with conception and design of the study, literature review and analysis, drafting and critical revision and editing, and final approval of the final version.
Conflict-of-interest statement: No potential conflicts of interest. No financial support.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Syuichi Koarada, MD, PhD, Department of Rheumatology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan. koarada@cc.saga-u.ac.jp
Telephone: +81-952-342367 Fax: +81-952-342017
Received: September 22, 2015
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
Abstract

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.

Keywords: Plasmablast, CD180, IgG4-related disease, Autoimmune disease

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.